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A   MANUAL 


V9 


DISEASES  OF  THE  NERVOUS  SYSTEM 


BY  THE   SAME  AUTHOR 

A  MartiSial  of  Diseases  of  the  Nervous  System. 

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Note. — The  illustrations  of  which  the  source  is  not  stated  are  original,  and 
part  of  the  copyright  of  the  hook. 


A   MANUAL 


DISEASES  OF  THE  NERVOUS  SYSTEM 


SIR  W.  R.  GOWERS,  M.D.,  F.R.C.P.,  F.R.S. 

CONSULTING  PHYSICIAN   TO   UNIVERSITY  COLLEGE  HOSPITAL  ;    PHYSICIAN  TO  THE  NATIONAL 
HOSPITAL   FOR   THE    PARALYSED   AND   EPILEPTIC,   QUEEN   SQUARE 


XTbtrt)  lEbition 

REVISED    AND     ENLARGED 

EDITED   BY 

SIR  W.  R.  GOWERS 

AND 

JAMES  TAYLOR,  M.A.,  M.D.,  F.R.C.P. 

SENIOR  ASSISTANT  PHYSICIAN  TO  THE  NATIONAL  HOSPITAL  FOR  THE  PARALYSED  AND  EPILEPTIC, 
QUEEN  SQUARE  ;    PHYSICIAN  TO  THE  NORTH-EASTERN  HOSPITAL  FOR  CHIL- 
DREN AND  TO  THE   NATIONAL   ORTHOPEDIC  HOSPITAL 


VOLUME    I 

DISEASES   OF  THE    NERVES   AND   SPINAL   CORD 
WITH  ONE  HUNDRED  AND  NINETY-TWO  ILLUSTRATIONS 


PHILADELPHIA 
P.    BLAKISTON'S    SON    &   CO. 

1012    WALNUT    STREET 
1907 


PREI^ACE  TO  THE  EIRST  EDITION. 


This  volume  contains  the  first  part  of  an  attempt  to  give  an 
account  of  diseases  of  the  nervous  system,  sufl&ciently  concise 
to  be  within  the  compass  of  the  time-pressed  student  or  busy 
practitioner,  and  yet  adequate  in  its  outline  of  a  subject  which 
has  become  wide  and  deep  beyond  any  other  part  of  medicine. 
Success  in  both  aims  can  scarcely  be  more  than  approximate. 

Most  of  the  illustrations  are  printed  from  blocks  prepared 
from  original  drawings  by  phototype  processes,  and  I  am  in- 
debted to  Messrs.  A.  and  W.  Dawson,  of  the  Typographic 
Etching  Company  (by  whom  most  of  the  work  has  been  done) 
for  the  care  they  have  taken  in  the  reproduction. 

The  casual  reader  may  perhaps  miss  subsidiary  letters  in  the 
illustrations  of  the  lesions  of  the  spinal  cord.  The  omission  of 
these  is  intentional.  A  knowledge  of  the  structural  topogi-aphy 
of  the  cord  is  the  first  requisite  in  the  study  of  its  morbid 
anatomy,  and  when  this  knowledge  is  gained,  the  figures  will 
be  understood  without  difficulty.  Familiarity  with  unlettered 
illustrations  facilitates  the  comprehension  of  sections  of  tha 
spinal  cord. 


Queen  Anne  Stbebt,  Lokdoi 
August,  1886. 


PREEAOE   TO  THE  THIED  EDITION. 


In  tlie  present  edition  every  chapter  has  been  subjected  to 
careful  revision,  and  numerous  additions  have  been  made  em- 
bodying the  results  of  personal  experience  and  the  more 
important  facts  ascertained  by  others. 

We  are  indebted  to  Dr.  Bertram  Abrahams  for  assistance  in 
the  revision  of  the  section  on  the  Anatomy  and  Functions  of 
the  Spinal  Cord,  and  to  Dr.  F.  E.  Batten  for  the  appendix  on 
the  Muscle-spindles. 

W.  R.  GOWERS. 
JAMES  TAYLOR. 


■January.  1890. 


CONTENTS. 


PAOB 

INTRODUCTION       .  .  .  .  .  .        1 

Classification  of  Diseases  of  the  Nervous  System,  2 


PAET  I.— GENERAL  SYMPTOMATOLOGY       . 

Symptoms  and  theie  Investigation  •  . 

Motor  Symptoms  •  .  .  , 

Inco-ordination  of  Movement,  10 
Sensory  Symptoms        .... 

Tactile  Sensibility,  13;  Sensibility  to  Pain,  15;  Sensibility 
to  Temperature,  15 ;  Muscular  Sensibility,  16 
Reflex  Action  .... 

Cutaneous     Eeflex    Action,    19;     Muscle    Reflex    Action- 
"  Ten  don -reflexes,"    20;    Paradoxical    Muscular    Contrac. 
tion,  27 
Changes  in  Nutrition  .  .  .  • 

Electrical  Irritability  .... 

MtrSCLES:   THEIR   ACTION   AND  PaEALTSIS 

Muscles  moving  the  Upper  Limb,  34 ;  the  Lower  Limb,  45 
Thb  General  Constitxjtion  op  the  Nervous  System  . 

PART  IL— DISEASES  OP  THE  NERVES 

General  Pathology  .... 

Structure  of  Nerves,  62 ;  Physiology  of  Nerves,  63 ;  Lesions  of 
Nerves,  Secondaiy  Degeneration,  65 

Symptoms  of  Neevb-injuey  and  Degeneeation 

Electrical  Changes,  71;  Sensory  Symptoms,  78;  Reflex  Action 
79  ;  Vaso- motor  and  Trophic  Disturbance,  79 

Injtteies  of  Neeves  .  .  .  . 

Compression,  80 

Inflammation  of  Neeves  :  Neiteitis  .  . 

Causes,  82 ;  Pathological  Anatomy,  83 ;  Symptoms,  85 ;  Dia^ 
gnosis,  87 ;  Prognosis,  87 ;  Treatment,  88 


7 

7 

13 


19 

27 
29 

33 
63 

62 

62 

71 

80 

82 


X  CONTENTS. 

Spinal  Meningitis  (continued) —  pagb 

Internal  Meningitis      .  •  •  •  .     313 

Acute  Internal  Meningitis        ....    313 

Causes,  313  ;  Pathological  Anatomy,  315  ;  Symptoms,  317  ; 
Diagnosis,  319  ;  Prognosis,  321 ;  Treatment,  321 

Chronic  Internal  Meningitis    ....     322 
Causes,  323  ;  Pathological  Anatomy,  324  ;  Symptoms,  328; 
Diagnosis,  330;  Prognosis,  332  ;  Treatment,  333 

H^MOREHAGE     INTO    SpiNAL     MeMBEANES  :     SpINAL     MENINGEAL 

HjiMOEEHAGE  :      H^MATOEEHACHIS  .  .  .      334 

Causes,   334;    Pathological   Anatomy,   335;    Symptoms,  336; 
Diagnosis,  337 ;  Prognosis,  338 ;  Ti-eatment,  338 

DISEASES  OF  THE   CORD  .  .  ,  .  .339 

Anemia  and  Hypee^mia  .  .  •  •  •    339 

Anaemia,  340 ;  Hypersemia,  342 

Inflammation— Myelitis  .       ■         .  .  •  ,    343 

Acute  Myelitis  .....     344 

Etiology,  344 ;  Pathological  Anatomy,  348 ;  Symptoms,  358 ; 
Varieties,  365;  Pathology,  367;  Diagnosis,  368;  Prog- 
nosis, 372  ;  Treatment.  373 

Abscess  ...•••    377 

Embolism  ......    377 

Cheonic  Myelitis  .....    378 

Etiology,  379  ;  Pathological  Anatomy,  382 ;  Symptoms,  386 ; 
Diagnosis,  387 ;  Prognosis,  388 ;  Treatment,  389 

COMPEESSION  ......      390 

Causes,  390 ;    Pathological   Anatomy,    390 ;    Symptoms,   393 ; 
Diagnosis,  394 ;  Prognosis  and  Treatment,  395 

Anteeioe  Polio-myelitis:  Ateophic  Spinal  Paealysis  •    395- 

Acute  Atrophic  Paralysis  [Infantile  Palsy)  .  .     396- 

Etiology,  397  ;  Symptoms,  399  ;  Pathology,  409 ;  Diagnosis, 
416  ;  Prognosis,  418  ;  Treatment,  419 
Subacute  and  Chronic  Atrophic  Spinal  Paralysis    .  .    424 

Acute  Ascending  Paealysis  ....    427 

Causes  and  Symptoms,  427,  428 ;  Pathology,  430 ;  Diagnosis 
and  Prognosis,  431 ;  Treatment,  432 

Paealysis    feom    Lessened    Atmosphekic    Peessuee — Dxvees' 

Paralysis — 'Caisson   Disease     ....    433 
Symptoms,  483  ;  Pathology,  434 

H^EMORRHAGE   INTO    SpINAL   CoRD — U^MATOMYELIA  .  .      437 

Etiology,  437  ;  Patholo.sical  Anatomy,  43.S ;   Symptoms,  439 ; 
Diiignosis,  441  ;  Prognosis  and  Treatment,  441 


CONTENTS.  XI 

FAGB 

DEGENERATIONS  OF  THE   CORD    .  •  •  -.442 

LocoMOTOE  Ataxy — Tabes  Dorsalis  .  .  .    444 

History,   444;   Causes,    445;    Symptoms,   448;    Motor,   449; 
Sensory,   450;    Eeflex,   454;    Eye,   455;    Sphincters,   458; 
Vaso-motor   and    Trophic,   459;    Visceral,  461 ;    Course   and 
Termination,   463 ;    Complications,  465 ;    Pathological   Ana- 
tomy,  467 ;    Pathology,  474 ;    Diagnosis,   480 ;    Prognosis, 
483 ;  Treatment,  484 
Pbimaey  Spastic  Paraplegia — Primary  Lateral  Sclerosis        .    48& 
General  Pathology,  489;  Causes,  492;   Symptoms,  493  ;  Patho- 
logical Anatomy,  497  ;  Diagnosis,  500;  Prognosis  and  Treat- 
ment, 502  ;  Family  form,  503 
Ataxic  Paraplegia  .  .  .  .  •     503 

Causes,   504;    Symptoms,    505;   Pathological   Anatomy,   507; 
Pathology,  508;   Diagnosis,  510;    Prognosis  and  Treatment, 
511 
Sclerosis  of  Coed  from  Toxic  Blood-states  •  >    511 

Pellagra        .  .  .  •  •  ^     511 

Causes  and  Symptoms  .  .  v.  .     512 

Sclerosis  from  other  Toxic  Blood-states       .  .  .     513^ 

Symptoms,  514;  Morbid  Anatomy,  515;  Pathology,  516; 
Diagnosis,  Prognosis,  Treatment,  618 
Hereditary  Ataxy — Friedreich's  Disease   .  .  .    519- 

Symptoms,  520 ;  Pathological  Anatomy,  525  ;  Pathology,  628; 
Diagnosis,  529 ;  Prognosis  and  Treatment,  530 
Simple  Senile  Paraplegia  ....    530 

Chronic  Spinal  Musculak  Atrophy:  Peogeessivb  Muscular 
Atrophy  ......     531 

Causes,   533;    Symptoms,   534;    Varieties   and   Complications, 
542,  543  ;  Course,  543  ;  Pathological  Anatomy,  545  ;  Path- 
ology, 551 ;  Diagnosis,  555  ;  Prognosis,  556  ;  Treatment,  557 
Arthritic  Muscular  Atrophy         .  .  .  ,     559 

Pathology,  560;    Diagnosis   and  Prognosis,  560;    Treatment, 

562 
Muscular  Atrophy  from  Over-use  •  .  663 

The  Muscular  Dystrophies  ....    563 

Pseudo-hypertrophio  Muscular  Paralysis :  Jjipomatous  Mus- 
cular Atrophy  .....     567 

Etiology,  568;   Symptoms,  569;   Varieties  and  Complica- 
tions,   576 ;    Pathological    Anatomy,   577 ;    Pathology, 
579  ;  Diagnosis,  581 ;  Prognosis,  582  ;  Treatment,  582 
Simple  Idiopathic  Muscular  Atrophy         .  .  .     583 

Causes,  585 ;  Symptoms,  586  ;  Pathological  Anatomy,  590 ; 
Diagnosis,  591 ;  Prognosis  and  Treatment,  592 
Peroneal  type  of  Family  Amyotrophy         .  .  .     593 

Family  Form  of  Muscular  Atrophy  in  Children  with  Spinal 
Cord  Lesion  .  .  .  .  •     597 

Muscular  Hyppvlrophy  •  •  >  .     599 


Xll  CONTENTS. 

FAOB 

Thomsen's  Disease:  Myotonia  congenita    •  •  .    601 

Pathology,  604 ;  Treatment,  605 ;  Congenital  Paramyotone«  605 ; 
Ataxic  Paramyotone,  606 

TUMOUES   OP  THE   CoED  .  .  .  .  .      607 

Etiology,  607  ;  Pathological  Anatomy,  608 ;  Symptoms,  616 ; 
Diagnosis,  622  ;  Prognosis,  625  ;  Treatment,  626 

Cavities  and  Fissttees  in  the  Coed:  Steingomtelia,  Htdeo- 
MTELiA,  Htdeoeehachis  inteena  .  .  .      628 

Etiological  Pathology,  629;  Symptoms,  639;  Diagnosis,  643; 

Prognosis  and  Treatment,  645 

Analgic  fanaeitium:  Moetak's  Disease      •  •  •    645 

Spina  bifida        .  .  ,  ,  .  •    647 

Teattmatic  Lesions  of  the  Coed    ....    652 
Causes,   652;    Pathology,    658;    Symptoms,  654;    Diagnosii, 
661 ;  Prognosis,  662  ;  Treatment,  663 

Functional  xsd  Nuteitional  Diseases  of  the  Coed  .    664 

Functional  Diseases,  664  ;  Nutritional  Diseases,  668 

APPENDIX  ON  MUSCLE-SPINDLES  •  .    671 

INDEX  .  .  .  .676 


LIST   OP   ILLUSTRATIONS. 


Fia.  pxoB 

1,  2,  The  Knee-jerk,  mode  of  obtaining  it               •  •  •21 

3.  The  Foot-clonus,  mode  of  obtaining  it                  •  •  •22 

4.  Tracing  of  the  Foot-clonus    .                 .                •  •  #23 

6.  6.  Paralysis  and  Wasting  of  the  Trapezius            .  •  •      34 

7.  Paralysis  and  Wasting  of  the  Deltoid  and  TmpeziuB  •  #35 

8.  Paralysis  of  the  Serratus  Magnus          .                .  •  .36 

9.  Paralysis  of  the  Deltoid         .                 ,                •  •  .36 

10.  Motor  Points  for  the  Arm,  inner  side  •               •  •  •      38 

11.  „                 „             outer  side   .                 .  •  •      39 

12.  Paralysis  of  the  Long  Kadial  Extensor  of  the  Wvist  •  #40 

13.  Contraction  of  the  Palmar  Fascia  .  .  •  ,41 
14t  Paralysis  of  part  of  the  Flexor  Digitorum  Sublimis  •  .41 
16 — 18.  Pai'alysis  of  the  Interossei  .  .  •  42,  43 
19,  20.  Claw-like  Hand  from  Nerve-injury                .  •  ,43 

21.  The  Thumb,  normal  position  of             .                •  .  ,44 

22.  Ape-like  Position  of  the  Thumb  from  Paralysis  of  its  Muscles  .      44 

23.  „  „  „  in  greater 
degree                 .... 

24.  Paralysis  of  the  Abductor  Brevis  and  Opponens  PoUicis 
26.  Motor  Points  on  the  Front  of  the  Thigh 

26.  „  of  Leg,  outer  side  .  . 

27.  „  on  the  Back  of  Thigh  and  Leg:       • 


28.  Talipes  Calcaneus  .... 

29.  „       Equinus  at  rest  and  in  Flexion  of  the  Ankle 

30.  „  „       extreme 

31.  Paralysis  of  the  Interossei  and  Muscles  of  the  Great  Toe 

32.  Diagram  of  Structure  of  Nerve- fibres  . 

33.  Degeneration  and  Regeneration  of  Nerve-fibres  . 

34.  Degeneration  of  Fibres  of  Cutaneous  Nerves 

35.  Chart  of  Degenerative  Reaction  in  transient  Nerve-injiiiy 

36.  „  „  in  permanent  degeneration 

37.  Tracings  of  Muscular  Contractions  in  Nerve  Degeneration 
38—43.  Chart  of  Electrical  Reactions  in  Neuritis      • 

44.  Neuritis,  Microscopical  Changes  .  • 

45.  Plexiform  Neuroma  from  the  Or,bit      ,  • 

46.  Neuromata  in  the  Skin  of  Abdomen      ,  • 

47.  „  Multiple  of  the  Arm  •  r 


75- 


44 
45 
46 
48 
49 
60 
51 
51 
61 
62 
66 
66 
71 
73 
74 
-77 
83 
91 
92 
92 


PAGE 


Bodies  and  to  the 


182 
194 
195 

200 
201 
202 
20:J 


SlY  LIST    OF    ILLUSTEATIOKS. 

Fig. 

4,8.  Neuromata,  Multiple,  of  the  Arm,  Nerves  dissected  out      .  -.92 

49.  Neuroma  of  the  Posterior  Tibial  Nerve                  ,                 ,  .92 

50.  „        of  Popliteal  Nerve  .                 .                .                .  .  92 

51.  Area  of  Anaesthesia  after  Division  of  8th  Cervical  and  1st  Dorsal 

Boots                .                .                .  .100 

52.  „                „           from  Disease  of  the  Circumflex  Nerve  .  104 
^3.  Wrist-drop  from  Paralysis  of  Musculo-spiral  Nerve              .  .  106 

54.  Carpal  Prominence  from  Paralysis  of  the  Extensors  of  the  Fingers    .  106 

55.  Palsy  of  Deltoid,  Supra-spinatus,  and  Infra-spinatus            .  .  112 
66,  57.  Contracture  of  Hand  from  a  growth  in  the  Cervical  Spine  .  112 

58.  Multiple  Alcoholic  Neuritis,  Palsy  of  Extremities                .  .  153 

59,  60.         „                 „          Microscopical  Appearances  in  the  Nerves  165,  166 
■61.  Apparatus  for  treatment  of  Drop-foot  . 

62.  Leprosy,  Areas  of  Anajsthesia 

63.  „        Changes  in  the  Nerves 

64.  The  Eelation  of  the  Vertebral  Spines  to  their 

Origin  of  the  Nerve-roots  .  .  ' 

65.  Diagram  of  a  Section  of  the  Spinal  Cord 

66.  Diagrams  of  the  Cord  at  different  levels 

67.  Nerve-cell  from  the  Spinal  Cord  of  Man 

68.  Nerve-cells  sending  a  Process  into  the  Anterior  Root           .  «  203 

69.  Junction  of  a  Divided  Nerve-fibre  with  separate  Nerve-cells  •  203 

70.  The  Groups  of  Nerve-cells  in  the  Anterior  Cornu                 ,  •  205 

71.  The  Elements  of  the  White  Substance  at  various  levels      •  •  208 

72.  Ascending  and  Descending  Degenerations             .                 •  .  210 

73.  Descending  Degeneration  in  Hemiplegia                               •  „'  211 

74.  Diagram  of  an  Element  of  the  Motor  Path          .                •  .  213 

75.  Degeneration  o£  the  Antero-lateral  Ascending  Tract            .  .  214 

76.  „                  „                    „                    „            and  the  Postero- 
median Column      ......  214 

77.  Degeneration  after  Injury  to  the  Cauda  Equina  .                «  214 

78.  „                 a  Lesion  in  Cervical  Region   .                 •  •  216 

79.  „                 Injury  to  the  lowest  part  of  the  Cord    •  .  216 

80.  Posterior  Cornu  and  Column  at  the  last  Dorsal  Segment     •  •  218 
SI.  Cervical  Section ;  Ascending  Degenerations          .                •  •  221 

82.  Central  Canal  in  Normal  Cords              .                 .                 .  ^  224 

83.  The  Arteries  of  the  Spinal  Cord             .                 .                •  .227 

84.  Spinal  Cord  contused  by  Gunshot  Injury              .                 •  •■  236 

85.  Tracings  of  the  Action  of  the  Sphincter  Ani         ,                •  .  246 

86.  Plan  of  the  Centre  for  Micturition        .                 .                 •  .  247 

87.  88.  Cutaneous  areas  corresponding  to  Sensory  Roots            •  250,  251 

89.  Fracture  of  the  First  Lumbar  Vertebra                 .                 •  •  283 

90.  Spinal  Cord  damaged  by  Fracture          .                 »                 •  •  284 

91.  Fracture  of  the  Fifth  Dorsal  Vertebra.                .                .  -284 

92.  Caries  of  the  Spine                  ..».,■■  288 

93.  External  Pachymeningitis  in  Caries  of  the  Spine                 .  311 
D4.  Purulent  Meningitis  ;  Section  of  Spinal  Cord  and  Membranes  316 

95.  „                ,,              Infiltration  of  Arachnoid  .                .  '^<    ' 

96.  Chronic  Alcoholic  Meningitis                 ,                ,                »  325 

97.  Myelitis ;  Inflammatory  Products         .                .                •  ,  349 

98.  Subacute  Myelitis,  Lumbar  Region      ,                .                •  .  350 


LIST    OP    ILLUSTRATIONS.  XV 

Fig.  i'AGE 

99.  Subacute  Myelitis,  more  liigVily  magnified  •  •  •    350 

100.  Subacute  Disseminated  Myelitis;  Spider  Cells   .  .  .     351 

101,  102.  Acute  Transverse  Myelitis ;   Dilatation  of  the  Central  Canal  362,  354 

357 
357 
361 
381 
381 
383 
385 
391 
392 
406 
406 
4^7 
4D7 
410 
411 
412 
456 
456 
459 
460 
461 
461 
467 
468 


103.  Ascending  Myelitis  from  Fracture  of  Spine        .  . 

104.  „  „         more  highly  magnified         •                • 

105.  Feet  in  Myelitis  of  Lumbar  Enlargement  •                 • 

106.  Chronic  Myelitis  ..... 

107.  Chronic  Sclerotic  Myelitis    .  .                 .                 . 

108.  „  „                 more  highly  magnified              . 

109.  Chronic  Meningo-myelitis ;  Syringomyelia         .  . 

110.  Compression  of  the  Spinal  Cord  and  Pressure-myelitis       . 

111.  Pressure-myelitis,  more  highly  magnified  .                 • 

112.  Atrophic  Spinal  Paralysis ;  Talipes  Valgus  and  Varus       . 

113.  „  „                 Talipes  Calcaneus    .                 . 

114.  „  „                Left  Arm                 .                • 

115.  „  „                affecting  the  Hand .                • 

116.  „  „                Microscopic  Changes               • 

117.  n  f>                 fourteenth  day         .                 . 

118.  „  „                Spinal  Cord  in  Lumbar  Eegioa 

119.  Unilateral  Tabetic  Ptosis     .... 

120.  Double  Tabetic  Ptosis  .... 

121.  Perforating  Ulcer  of  the  Foot  in  Tabes  .                . 
122  Locomotor  Ataxy  ;  Swelling  of  Elbow                .                • 

123.  „  „        Retroflexion  of  Knee-joints .                • 

124.  Tabetic  Foot  ..... 

125.  Locomotor  Ataxy ;  Sections  of  Spinal  Cord        .  . 

126.  „  „         Section  of  Cervical  Region   .                 . 

127.  128.  „  „        complete  Sclerosis  of  Posterior  Columns          469,  470 
129  „               „         Chronic  Meningitis                .                 .                 .     472 

130.  Osseous  Lesions  in  Tabes     .....     473 

131.  Congenital  Spastic  Paralysis  (Cerebral)  .                 .                •    496 

132.  ,,  „                    „           Cross-legged  Progression     •    496 

133.  Sclerosis  of  the  Pyramidal  Tracts        ....    497 

134.  „  „                 in  Muscular  Atrophy     .                .    498 

135.  Ataxic  Paraplegia;  Combined  Lateral  and  Posterior  Sclerosis  •    608 

136.  Cord  in  Pellagra  ......     612 

137.  Sclerosis  of  Cord  in  Anaemia  ....     615 

138.  Lateral  Curvature  in  Hereditary  Ataxy  ...     522 

139.  Deformity  of  Foot  in  „                             ...     523 

140.  Hereditary  Ataxy ;  Sections  of  Lumbar  Cord    .  •                .     525 

141.  „  „       Sections  of  Spinal  Cord       .                 .                .     523 

142.  „  „                „         Cervical,  Doisal,  and  Lumbar  Regions     526 

143.  „  „        Degeneration  of  Posterior  Nerve-roots                .     527 

144.  Progressive  Muscular  Atrophy  :  Wasting  of  Back  and  Arms  .     636 

145.  „  „                   Wasting  of  the  Back  Muscles         .    636 

146.  „  „                    Weakness  of  the  Neck     .                 .     637 
147—150.  „  „                   Fibres  of  Wasted  Muscles               .     546 

151.  Normal  Anterior  Cornu        .....    647 

152.  Progressive  Muscular  Atrophy ;  Anterior  Cornu  .                ,     547 

153.  „  „                  Degeneration     of      the     Anterior 

Cornu  and  Pyramidal  Tracts      .     648 


XVI  LIST   OF    ILLUSTRATIONS. 

Fig.  pagb 

154.  Progressive  Muscular  Atrophy  ;  Degeneration  in  Medulla  and  Cord  549 

155,  156.  Pseudo-hypertrophic  Paralysis    ....  570 

157.  „                       „             Partial  Enlargement  of  Eectus  .  570 

158,  159.  If  M  Absence  of    Latissimus   Dorsi ; 

Enlargement  of  Infraspinatus  571 

160,  161.             „                       M             Mode  of  rising            .                .  572 

162.  „  „  Late  Stage ;  Muscular  Contrac- 

tion and  Wasting    .                 .  574 

163.  Pseudo-hypertrophic  Paralysis ;  Weakness  of  the  Spinal  Muscles     .  575 

164.  „                        „            Enlargement  of  the  Vasti                 .  576 
1(J5,  166.         „                        „            Muscular  Alterations        .             677, 578 

167.  Section  of  a  Myolipoma  attached  to  the  Spinal  Cord          •                .  680 

168.  Simple  Idiopathic  Muscular  Atrophy.                   .                 .                 •  684 
169 — 171.  Idiopathic  Muscular  Atrophy  affecting  the  Face      .                .  589 

172.  „                     „         showing  Lordosis     .                 .                 •  690 

173.  Advanced  Muscular  Atrophy,  probably  of  the  "  Peroneal  Type"     .  595 

174.  Myolipoma  of  the  Spinal  Cord              ....  608 

175.  Tumour  growing  from  the  Dura  Mater               .                .                .  611 

176.  Sarcoma  between  the  Arachnoid  and  Pia  Mater  .               •               .  611 

177.  178.  Tumour  growing  from  the  Dura  Mater       .  •  611,  612 

.  612 

.  612 

.  613 

.  614 

.  630 
.681 

with  extensive  Gliomatosis          .                 .                •  632 


179.  „         of  the  Cauda  Equina 

180.  Neuromata  of  the  Cauda  Equina 
181..  Central  Tumour  of  Spinal  Cord 

182.  Syphiloma  in  the  Spinal  Cord 

183.  Central  Canal  in  Normal  Cords 

184.  Syringomyelia  from  a  Child 


185. 
186. 
187. 
188 
189, 


with  Tumours  of  the  Pons  and  Cauda  Equina  •  633 

aflsociated  with  Multiple  Growths               .  •  635 

with  Hsemorrhiii^e  into  the  Cavity              .  •  636 

with  Irregular  Cavities  in  the  Spinal  Cord  •  639 

190.  Spina  Bifida ;  sections  of  Spinal  Cord                  .                »  •  649 

191.  Diagram  of  Muscle-spindle                   .                .                •  •  672 

192.  Transverse  Sections  of  Normal  Muscle-spindl*                  »  •  673 


DISEASES  OF  THE  NERVOUS  SYSTEM. 


INTRODUCTION. 

A  CLASSIFICATION  of  Diseases  of  tlie  Nervous  System,  at  once 
scientific,  exact,  and  conrenient  for  systematic  description,  is  not  at 
present  possible.  If  we  attempt  to  classify  the  diseases  according  to 
either  seat  or  nature,  we  are  met  at  once  by  difficulties  wMcli  prevent 
any  complete  arrangement.  These  difficulties  arise  from  the  fact  that 
many  diseases  affect  more  than  one  part  of  the  nervous  system,  that 
the  precise  seat  of  other  maladies  is  unkaown,  and  that  we  are  still 
ignorant  of  the  nature  of  a  considerable  number  of  diseases.  A  rough 
division  is  often  made  into  two  broad  classes  of  "organic"  and 
"functional"  disease.  The  first  class,  that  of  "organic  diseases," 
comprehends  those  in  which  there  is  always  a  visible  lesion,  manifesting 
sometimes  the  morbid  process  which  constitutes  the  disease,  sometimes 
only  the  ultimate  result  of  that  process.  The  second  class,  that 
of  "  functional  diseases,"  is  less  definite,  comprehending  (1)  those 
diseases  that  consist  only  in  a  disturbance  of  function,  and  are  there- 
fore properly  so  designated ;  and  (2)  many  diseases  which  have  this  in 
common  with  true  functional  disease,  that  they  are  transient  and  not 
permanent,  and  that  they  are  not  known  to  depend  on  organic  changes. 
But  there  is  also  a  large  class  of  diseases  in  which  no  constant  lesion 
has  been  discovered,  but  which  are  not  transient,  and  cannot  well  be 
placed  in  either  of  these  classes.  This  simple  classification  is  therefore 
inadequate.  It  may  be  well  to  consider  in  what  respect  it  needs 
alteration  to  meet  the  requirements  of  our  present  knowledge. 

The  term  "organic  disease,"  as  commonly  used,  means  visible 
disease.  But  visibiUty  is  relative ;  it  depends  on  the  means  of  vision. 
The  application  of  the  microscope  to  pathology  immediately  increased 
the  range  of  organic  disease,  and  a  similar  increase  has  resulted,  in  our 
own  day,  from  the  use  of  staining  agents,  which  render  visible  many 
changes  that  could  not  before  be  seen.  Still  it  remains  true  that  the 
degree  of  alteration  determines  visibility.  Molecular  changes  in 
nutrition,  considered  as  such,  must  be  colossal  to  be  detected.  Such 
VOL.  I.  1 


2  INTRODUCTION. 

alterations,  not  sufficient  to  be  seen,  but  still  considerable,  probably 
constitute  the  morbid  process  in  many  diseases  that  are  commonly 
classed  as  "  functional."  Hence  we  may  distinguish  a  class  of  "  nutri- 
tional diseases  "  as  to  a  large  extent  distinct  from  those  that  consist 
in  a  mere  derangement  of  function.  While  distinguishing  these  t"wo 
classes,  we  cannot  entirely  separate  them ;  nutrition  and  function  are 
mutually  dependent,  and  prolonged  functional  disturbance  must  deter- 
nune  nutritional  change. 

Visible  disease  varies  much  in  nature  and  degree.  Some  forms  can 
always  be  seen  with  the  unassisted  eye;  they  change  the  shape  or 
colour  or  consistence  of  the  part.  Other  forms  can  be  detected  only 
with  the  aid  of  the  microscope ;  while  many  may  be  seen  with  the 
unassisted  eye  when  their  degree  is  considerable,  and  only  by  means 
of  the  microscope  in  their  early  stage  or  when  in  slight  degree.  It  is 
convenient  to  term  such  alterations,  the  visibility  of  which  is  alto- 
gether a  matter  of  degree,  "  structural  diseases."  Thus  we  may  dis- 
tinguish four  pathological  classes  of  disease  of  the  nervous  system : 

1.  Organic  disease  (or  "  coarse "  organic  disease)  ;  such  as  tumour, 
haemorrhage,  softening. 

2.  Structural  disease ;  such  as  most  forms  of  sclerosis. 

3.  Nutritional  disease;  such  as  general  paralysis  of  the  insane, 
paralysis  agitans. 

4.  Functional  disease ;  such  as  reflex  convulsions,  and  many  forms 
of  hysteria. 

In  the  first  class  the  morbid  process  always  begins  outside  the 
nerve-elements  themselves.  In  the  second  class  it  may  begin  in  them 
or  outside  them.  In  the  third  and  fourth  classes  these  elements  are 
probably  always  primarily  affected. 

Such  a  classification  helps  us  to  obtain  clearer  views  of  the  primary 
relations  of  disease,  but  is  not  convenient  for  systematic  description. 
For  this  a  hybrid  system  is  necessary,  in  which  organic  and  structural 
diseases  are  classified  according  to  their  seat, — in  the  nerves,  spinal 
cord,  or  brain.  With  each  group  may  be  placed  some  nutritional  or 
functional  maladies  of  local  incidence.  The  bulk  of  these  diseases  are, 
however,  best  considered  separately,  after  the  organic  diseases  have 
been  described,  since  many  of  them  are  of  wide  distribution  or 
uncertain  seat. 

Classification  has  two  purposes  to  serve.  It  serves  to  bring  out  the 
differences  in  the  nature  of  disease,  and  thus  to  define  our  conceptions 
and  make  clear  our  distinctions.  But  this  purpose  is  less  obtrusive 
than  the  second, — to  furnish  us,  as  it  were,  with  shelves  on  which  to 
place  our  conceptions,  and  labels  with  which  to  ticket  them,  so  that 
we  may  find  them  when  we  need  them  and  study  them  in  a  useful 
order  with  the  least  loss  of  time. 

Even  if  our  knowledge  were  perfect,  it  is  not  likely  that  any  scientific 


INTRODUCTION.  3 

classification  founded  on  natural  distinctions  would  serve  the  prac- 
tical needs  of  an  arrangement  for  use.  Our  present  knowledge  is  un- 
questionably so  very  far  from  perfect  as  to  be  inadequate  even  for  a 
complete  natural  classification ;  wliile  an  attempt  to  construct  one  of 
this  character  that  shall  be  also  of  practical  convenience  is  a  mere 
waste  of  time.  For  use,  we  must  divide  where  we  ought  only  to 
distinguish ;  and  we  have  to  place  near  together,  for  convenience'  sake, 
maladies  that  should  be  not  only  far  from  each  other,  but  sometimes  far 
from  all  others. 

Yet  it  is  useful  to  attempt,  if  not  to  classify,  at  least  to  distinguish  the 
chief  pathological  groups  of  the  morbid  processes  that  are  commonlj 
regarded  as  diseases  of  the  "  nervous  system,"  and  are  so  designated 
Unserviceable  as  a  practical  division,  the  attempt  affords  a  general  vier*.' 
of  the  nature  of  the  maladies,  including  the  various  influences  to  whioa 
they  are  due,  and  therefore  by  which  their  nature  is  determined.  The 
view  that  is  obtained  is  not  less  usef  id  because  the  groups  that  are,  in 
the  mass,  well  defined,  are  perceived  to  have  connecting  links  when 
their  borders  are  scrutinised,  or  are  even  found  here  and  there  to  blend 
in  places  where  a  demarcation  can  only  be  artificial.  We  perceive  thus 
that  elements  are  common  to  more  than  one,  and  such  elements  may  be 
thus  more  clearly  discerned,  and  their  importance  is  more  ckarly  re- 
vealed. 

The  first  great  distinction,  which  is  indeed  a  cleavagG  running 
through  all  the  varieties  distinguished  for  practical  convenience,  is 
that  of  primary  seat.  Some  diseases  begin  in  the  nerve-elements,  others 
outside  them,  in  other  structures.  These  structures  have  widely 
different  pathological  relations.  The  bones  which  protect,  the  fibrous 
tissues  that  invest  and  support,  the  vessels  which  convey  the  blood 
to  or  from  the  nerve-structures,  bring  the  diseases  of  these  structures 
into  connection  with  most  chronic  and  acute  constitutional  diseases ; 
the  blood  itself,  altered  by  numerous  and  widely  different  affections, 
frequently  produces  disturbance  of  function  or  of  structure,  and 
multiphes  the  relations  of  disease  of  the  nervous  system  to  an 
extent  that  is  to  us  indefinite,  and  potentially  is  all  but  infijiite. 
Not  only  does  the  blood  cause  disease  by  the  direct  influence  of  its 
changed  constitution  or  the  morbid  agents  it  bears,  but  the  physical 
conditions  of  the  circulation,  by  excess  or  deficiency  of  force,  and  the 
passage  in  it  of  bodies  that  can  obstruct  the  arteries,  involve  a  direct 
relation  to  morbid  states  of  the  heart,  which  is  frequently  obtrusive. 

The  affections  which  constitute  connecting  links  between  the  two 
general  classes  of  disease,  those  in  which  the  nerve-structures  suffer 
primarily  and  suffer  secondarily,  deserve  a  general  notice.  Some  of 
them  present  to  view  certain  pathological  features  which  are  of  great 
importance,  and  which  we  have  only  lately  become  able  to  perceive  dis- 
tinctly if  not  yet  clearly. 


4  LNTEODUCTION. 

The  variotis  morbid  growths  are  also,  for  the  most  part,  diseases 
only  in,  not  of,  the  nervous  system,  although  one  variety  of  neoplasm 
affords  another  illustration  of  the  connection  between  the  two  classes ; 
gliomata  connect  extrinsic  growths  with  diseases  that  are  primarily  of 
the  nerve- structure.  The  supporting  neuroglia,  different  from  the 
nerve- structures  as  it  seems,  and  indeed  is,  consists  of  a  residue  of  the 
embryonal  tissue  from  which  the  nervous  elements  were  developed.  In 
its  nutritional  tendencies  it  presents  a  relation  to  the  nerve- structures 
which,  though  definite,  is  opposite ;  it  overgrows  when  the  latter  decay, 
and  thus  gives  rise  to  the  condition  of  secondary  "  sclerosis."  Its 
growth  seems  normally  to  be  restrained  by  the  vitahty  of  the  higher 
tissue,  and  becomes  exuberant  as  soon  as  that  of  the  latter  is  lessened. 
It  may  persist  in  tracts,  large  or  small,  when  there  is  a  congenital 
arrest  of  development,  such  as  gives  rise  to  the  cavities  called  "  syringo- 
myeha ; "  this  persistence  is  especially  important  because  the  tissue 
may  cease  to  be  quiet,  may  grow  and  develop  into  the  definite  and 
dangerous  condition  kaown  as  "  central  gliomatosis."  But  the  neuroglia, 
as  the  "  connective  tissue  "  of  the  central  structures,  shares  the  lower 
morbid  tendency  of  the  interstitial  tissue  of  all  organs.  It  is  by  an 
overgrowth  of  tlois,  in  random  foci,  pi'esenting  at  first  some  of  the 
features  of  inflammation,  that  "  insular  sclerosis  "  develops,  while  the 
neuroglia  also  takes  a  chief  part  in  all  forms  of  interstitial  inflammation 
in  the  brain,  cord,  and  nerves.  Its  secondary  overgrowth  in  degenera- 
tion of  the  nerve-structures  varies  in  amount  and  in  energy ;  even 
when  secondary,  it  seems  occasionally  to  assume  an  independence  which 
makes  it  excessive,  and  an  energy  which  involves  some  of  the  features 
of  inflammation. 

We  have  to  distinguish  from  the  primary  interstitial  changes,  those 
acute  processes  in  which  the  nerve-structures  suffer  primarily,  but  with 
rapidity.  When  they  undergo  slow  decay  we  call  it  "  degeneration," 
but  an  acute  destructive  change  may  take  place  so  rapidly  as  to  resemble 
"parenchymatous  inflammation" — i.  e.  inflammation  in  which  the 
primary  element  is  in  the  proper  functional  structures  of  an  organ. 
Such  changes  belong  to  the  group  of  diseases  that  are  truly  of  the 
nervous  system,  but  are  not  always  readily  distinguished  from  the  inter- 
stitial inL^mmations,  since  the  interstitial  tissue  and  vessels  may  parti- 
cipate in  the  more  intense  processes.  Their  course  presents  aU 
gradations,  from  the  most  acute  to  chronic  forms,  but  even  in  the 
former  it  is  often  remarkable  how  small  is  the  share  the  connective- 
tissue  elements  take.  These  processes  have  become,  of  late,  very  im- 
portant, on  account  of  the  extent  and  degree  to  which  they  can  be 
traced  to  toxic  influences.  They  constitute,  in  slow  form,  the  systemic 
degenerations  of  the  central  nervous  system,  and  also,  in  both  slow  and 
rapid  forms,  the  varieties  of  toxic  peripheral  "  neuritis."  The  former, 
as  well  as  the  latter,  have  been  extensively  traced  to  the  influence  of 
blood-states.  But  as  chronic  "degeneration,"  they  are  also  often  due 
to  defective  vitality,  or  imperfect  vital  endurance,  senile  or  premature, 


INTRODUCTION.  5 

occurring  in  late  life  before  otter  structures  decay,  or  as  isolated 
failure  soon  after  development  is  complete.  The  latter  class  is  represented 
by  the  diseases  of  which  "  hereditary  ataxy  "  is  a  type. 

The  toxic  influences  -which  are  thus  effective  seem,  so  far  as  we  can 
trace  their  nature,  to  be  chemical.  They  may  be  metallic  poisons — or 
the  simpler  chemical  substances  of  organic  origin,  as  alcohol  — or  com- 
plex organic  compounds,  either  received  from  without  or,  more  often, 
formed  within  the  body.  They  are  often  formed  within  the  body  by 
the  organisms  that  give  rise  to  "  acute  specific  diseases,"  as  has  been 
clearly  proved  in  the  case  of  diphtheria ;  to  the  same  mechanism 
many  nervous  sequelse  of  acute  diseases  are  apparently  due.  These 
poisons,  especially  those  of  organic  origin,  present  the  remarkable 
feature  of  a  special  tendency  to  influence  certain  nerve-structiu"es,  that 
we  are  only  able  to  distinguish  from  others  by  their  difference  of 
function  ;  underlying  tliis  must  be  some  minute  difference  of  consti- 
tution which  renders  them  more  susceptible  to  the  particular  chemical 
compounds.  These  structures  suffer  most  where  their  vitality  is  least, — 
for  instance,  the  ends  of  the  uei^e-fibres, — the  parts  farthest  from  the 
nerve-cell  of  which  they  are  the  processes.  Thus  many  toxic  agents  act 
on  the  extremities  of  the  long  sensory  or  motor  nerves  in  the  limbs, 
causing  "  degenerative  peripheral  neuritis,"  while  a  similar  degeneration 
of  the  fibres  of  the  pyramidal  tracts  of  the  spinal  cord  (the  processes 
of  the  motor  cells  of  the  cortex)  constitutes  "  lateral  sclerosis."  The 
motor  fibres  of  the  limbs  are  processes  of  the  cells  of  the  anterior  grey 
matter  of  the  cord ;  the  sensory  fibres  proceed  from  the  cells  of  the 
ganglia  in  the  posterior  nerve-roots.  Of  the  latter,  those  which  seem 
most  prone  to  suffer  are  the  afferent  fibres  from  the  muscles.  In  loco- 
motor ataxy  these  nerves  suffer  fii-st,  apparently  from  a  toxic  agent 
which  is  a  result  of  syphilis,  and  acts  also  on  the  other  processes  of 
these  ganglion-cells  — those  which  become  the  fibres  of  the  posterior 
median  columns.  These  ascend  the  cord,  but  depend  for  their  vitality 
on  the  cells  of  the  posterior  ganglia.  Thus  the  degeneration  in  the 
spinal  cord,  the  "  posterior  sclerosis,"  and  the  changes  in  the  peripheral 
nerves,  are  but  parts  of  the  toxic  effect  on  one  set  of  "neurons." 
The  afferent  nerves  from  both  skin  and  muscle  suffer,  moreo\^er, 
from  many  other  toxic  causes,  and,  alike  in  alcoholic  neuritis  and  in 
diphtheritic  palsy,  we  may  have  a  close  resemblance  to  locomotor  ataxy. 
A  corresponding  lesion  underlies  the  correspondence  of  symptoms. 
The  special  susceptibility  of  the  muscle  nerves  is  the  cause  of  the 
frequency,  and  early  date,  of  the  loss  of  the  knee-jerk,  and  of  the 
significance  of  the  symptom. 

Other  toxic  agents  influence,  not  the  peripheral  ends  of  the  chief 
processes  of  the  cells,  but  the  structures  in  which  nerve-energy  is 
produced  in  the  central  mechanism.  Such,  for  instance,  is  strychnine, 
and  the  strychnine-like  products  of  the  organism  of  tetanus  ;  while 
one  element  in  the  production  of  the  cui-ious  disturbance  of  chorea 
seems  to  be  the  action  on  the  elements  of  the  motor  cortex  of  some 


6  INTRODUCTION. 

poison  allied  to,  but  not  identical  witli,  that  which  causes  acute 
rheumatism,  and  is  probably  a  complex  organic  chemical  compound. 

Eelative  deficiency  in  the  power  of  resisting  toxic  agents  must  be 
ascribed  to  imperfect  vital  energy,  a  lower  degree  of  the  energy  of  life 
that  maintains  nutrition.  Such  imperfection  is  manifested  by  a  class 
of  cases  in  which  failure  of  nutrition  occurs  spontaneously  ia  certain 
structures,  either  in  late  life,  as  in  many  cases  of  senile  muscular 
atrophy,  or  labio-glossal  paralysis,  or  senile  braia  failure,  or  paralysis 
agitans, — or  else  soon  after  complete  development  is  achieved,  as  in 
the  so-called  "  hereditary  ataxy "  and  its  congeners.  Moreover  it 
is  possible  that  a  slight  difference  in  the  power  of  vital  endurance  in 
some  structures,  causes  a  greater  readiness  to  suffer  degenerative 
changes  in  middle  life  under  acquired  influences. 

The  toxic  agents  which  can  thus  act  on  the  nerve-structures  and 
impair  their  nutrition  and  function  are  numerous  and  varied.  They 
may  be  derived  directly  from  without,  may  be  produced  within  the 
body  by  the  organisms  of  disease,  and  may  be  produced  by  disordered 
chemical  processes  apart  from  external  influence,  or  by  an  external 
influence  that  becomes  effective  through  a  peculiar  predisposition.  They 
present  relations  to  those  rheumatic  poisons  which  act  on  the  fibrous 
tissues  and  on  other  stnictures.  Exposure  to  cold,  apparently  the  same, 
may  cause  in  different  persons — a  general  catarrh,  local  inflammation 
(as  tonsillitis),  acute  rheumatism  (with  or  without  endocarditis),  spinal 
myelitis,  or  peripheral  neuritis,  apparently  according  to  the  nature  of 
the  toxic  agent  generated,  determined  by  the  constitution  of  the  indi- 
vidual. A  slight  difference  in  the  initial  effect  may  determine  a 
divergence  in  the  chemical  processes  under  the  influence  of  life,  and 
the  ultimate  result  is  the  same  in  no  two  individuals,  and  may  be  very 
different. 


GENERAL   SYMPTOMATOLOGY. 


SYMPTOMS  AND  TSUIR  INfTESTiaATION. 

The  nervous  system  is  almost  entirely  inaccessible  to  direct  obser- 
vation. The  exceptions  to  tliis  are  trifling:  the  termination  of  one 
nerve,  the  optic,  can  be  seen ;  some  of  the  nerve- trunks  in  the  limbs 
can  be  felt,  either  in  the  normal  state  or  when  enlarged  by  disease.* 
As  a  rule,  the  state  of  the  nervous  system  can  be  ascertained  only  by 
the  manner  in  vs^hich  its  vrork  is  done,  and  morbid  states  reveal  their 
presence  by  the  derangement  of  function  which  they  cause. 

The  functions  of  the  nervous  system  are  often  divided  into  mental, 
motor,  and  sensory  ;  but  they  are  far  more  extensive  than  those  words 
denote.  The  functions  of  all  the  organs  of  the  body,  all  states  of  the 
blood-vessels  and  hence  of  the  circulation  within  them,  and  even  all 
processes  of  nutrition,  are  under  the  control  of  the  nervous  system. 
The  disturbance  of  nerve-function  produced  by  disease  is  conterminous 
in  extent  with  the  human  organism.  Our  knowledge  of  these  symptoms 
is  constantly  increasing  in  range  and  in  degree.  They  differ,  however, 
very  much  in  relative  importance.  Some  occur  more  frequently  than 
others,  and  are  more  direct  in  significance.  Many  have  a  special 
relation  to  certain  parts  of  the  nervous  system,  while  others  occur  in 
diseases  of  all  parts.  It  may  be  well,  at  the  outset,  to  describe  the 
characters  of  some  of  the  most  frequent  symptoms ;  while  the  more 
special  symptoms  can  be  conveniently  considered  when  we  deal  with 
diseases  of  that  part. 

MoTOB  STMPTOMS.f — ^Disturbance  of  motion  is   one  of  the  most 

•  To  the  new  X  rays,  unfortunately,  the  nerve-centres  are  opaque  and  in:icce— 
sible,  and  the  nerves  differ  too  little  in  transpHrency  from  the  other  ti-snes  to 
permit  them  to  be  seen,  while  their  course  is  seldom  such  as  to  bring  tliem  into 
conspicuous  distinction  from  otlier  structures. 

+  Some  modification  of  the  word  kinesis  (akinesis,  &c.)  is  sometimes  used  to 
designate  motor  symptoms.  These  words  seem  to  me  to  add  nothing  to  our  know- 
ledge or  to  its  clearness — certainly  nothing  to  compensate  for  their  cumbersome 
character  J  and  the  definite  additional!  new  word-^  make  to  the  infonnati'ai  tliiit  has 
to  be  acquired  is  not  always  sufiiciently  weighed  against  their  supposed  advantage. 


8  SYMPTOMS. 

common  effects  of  disease.  Movement  is  produced  by  muscles,  but 
the  muscles  are  excited  to  contraction  by  the  nervous  system.  Most 
alterations  of  motility,  both  defect  and  excess,  are  due  to  its  derange- 
ment. The  conditions  are  very  few  in  which  motor  changes  originate 
in  the  muscles  themselves.  Defect  of  movement  is  of  muscular  origin 
only  in  some  cases  of  primary  alteration  in  the  nutrition  of  the 
muscles.  When  there  is  a  general  impairment  of  nutrition,  the 
muscles  participate  in  this ;  they  are  ill-nourished,  and  therefore  feeble. 
Such  a  condition  is  met  with  in  chronic  wasting  diseases,  in  anaemia, 
and  as  an  acute  affection  in  all  acute  febrile  diseases.  This  is  Ihe 
chief  cause  of  the  muscular  weakness  that  so  often  results  from  fevers ; 
it  may  even  proceed  to  the  degree  of  causing  visible  changes  in  the 
aspect  of  some  fibres. 

In  a  few  chronic  diseases  the  muscles  undergo  a  primary  altera- 
tion. One  is  the  disease  known  as  "  pseudo-hypertrophic  paralysis," 
a  special  form  of  a  wider  malady,  in  which  there  is  atrophy  of  the 
muscles,  due  to  their  own  defective  power  of  vital  maintenance.  Rheu- 
matic and  other  inflammations,  commonly  interstitial,  may  greatly 
impair  their  power,  first  by  the  pain  contraction  causes,  and  then  by 
damage  to  the  nerves  in  this  tissue,  a  state  to  be  distinguished  from 
the  primary  affection  of  these  endings  themselves.  In  almost  all  other 
conditions,  loss  of  power  in  the  muscles  is  dependent  on  the  state  of 
the  nervous  system,  either  directly  or  through  defective  nutrition  of 
the  muscles,  or  both.  The  nutrition  of  the  muscular  fibres  depends  on 
the  motor  nerves,  although  we  are  still  ignorant  of  the  precise  nature 
of  that  dependence.  The  subject  will  be  referred  to  again  in  the 
special  account  of  the  changes  that  result  from  injuries  to  the  nerves. 

So  also  with  increased  and  involuntary  muscular  action.  We  know 
little  of  what  has  been  termed  "  idio-muscular  contraction," — that 
is,  contraction  originating  in  the  muscle  itself.  Whenever  the  whole 
of  a  muscle  contracts,  the  contraction  is  due  to  an  influence  acting 
on  aU  the  fibres,  and  this  is  generally  from  the  nerve-centres.  Even 
muscular  "tone"  is  dependent  on  the  connection  of  the  muscle 
with  the  nerve-centre,  since  it  ceases  when  the  connection  is  de- 
stroyed. It  is  possible  that  the  extreme  degree  of  the  contraction  in 
cramp  is  the  effect  of  a  tendency  in  the  muscle  itself.  When  the 
shortening  exceeds  a  certain  degree  it  becomes  fixed  in  a  peculiar 
manner,  which  seems  as  if  it  were  due  to  a  tendency  in  the  fibres  to 
pass  beyond  the  power  of  relaxation.  Contraction  of  the  whole  of  a 
muscle  may  also  result  from  mechanical  influences  acting  on  every 
part  equally,  as  in  traction.  But  these  exceptions  occur  under  special 
conditions. 

The  partial  contractions  that  are  termed  "  fibrillary  "  are  probably 
local,  since  they  are  so  readily  produced  by  mechanical  stimulation, 
such  as  a  tap  on  the  muscle.  Yet  fibrillary  contractions,  although 
sometimes  local,  may  be  often  of  central  origin.  This  is  also  true  of 
the   flickering  contractions   that   are  so   common  in  persons  whose 


MOTOR.  9 

nervous  system  is  enfeebled,  the  "  live  flesh "  of  popular  language. 
They  may  occur  in  any  muscle,  from  the  orbicularis  palpebrarum  (in 
which  they  are  most  common)  to  the  gluteus  maximus.  After  damage 
to  the  nerve-fibres  to  the  muscle  they  occur  in  special  degree ;  they  are 
often  frequent  for  years  in  the  calf  muscles  after  sciatic  neuritis. 

Paralysis. — Defect  of  movement  may  present  every  degree  from 
slight  weakness  to  absolute  loss.  There  may  be  inability  even  to  make 
the  muscle  contract,  or  the  voluntary  contraction  may  occur,  but  be  too 
feeble  to  move  the  parts  to  which  the  muscle  is  attached.  The  latter 
difference  is  to  some  extent  relative,  since  it  varies  with  the  readiness  with 
which  the  part  can  be  moved — i.  e.  with  the  resistance  to  be  overcome. 
A  contraction  that  will  flex  a  finger  may  be  insufficient  to  flex  the  wrist. 
The  difference  may  vary  in  the  same  part  according  to  position.  A 
degree  of  power  in  the  flexors  of  the  hip,  that  will  move  the  leg  when 
the  patient  is  lying  on  his  side,  may  be  insufficient  to  do  so  when  the 
patient  is  lying  on  the  back.  This  difference  is  even  more  conspicuous 
in  some  other  muscles  ;  paralysis  of  the  extensors  of  the  elbow  may  be 
discovered  only  on  an  attempt  to  raise  the  hat:  in  many  positions 
extension  is  due  to  the  weight  of  the  arm,  much  more  than  to  the 
muscles,  so  that  considerable  weakness  may  be  unobserved. 

The  term  "  paralysis  "  has  long  been  applied  to  all  degrees  of  defect 
of  power,  a  distinction  being  made  between  complete  and  incomplete 
paralysis.  Partial  loss  of  power  is  also  sometimes  termed  "  paresis." 
The  advantages  of  "paresis"  over  the  word  "weakness"  are  chiefly 
psychical,  and  depend  on  the  popular  horror  of  "  paralysis,''  which  has 
become  specially  associated  with  complete  loss  of  power,  and  the  idea 
of  incurability  seems  inseparably  attached  to  the  term. 

Our  means  of  estimating  defects  of  muscular  contraction  are  very 
imperfect,  in  consequence  of  the  peculiar  and  various  form>'  of  move- 
ment which  are  produced.  An  instrument  to  measure  the  f  o»^e  exerted 
is  called  a  "  dynamometer,"  but  any  simple  form  of  insti-Mment  can 
have  only  limited  application.  Several  have  been  devised,  tut  that  in 
general  use  consists  of  an  oval  steel  ring,  which  can  be  narrowed  either 
by  compression  applied  to  its  smaller  diameter  or  by  tractioxi  applied 
to  its  longer  diameter.  The  change  in  the  shape  of  the  dyns^-mometer, 
and  therefore  the  force  exerted,  is  shown  by  the  movement  of  an 
index  on  a  scale  that  has  a  double  graduation,  a  closer  for  com- 
pression, which  is  easy,  and  a  wider  for  traction  lengthways,  which 
is  difficult.  This  form  of  dynamometer  is  known  as  "  Charriere's," 
from  the  name  of  its  first  maker.  Another  less  convenient  form 
was  devised  by  Duchenne.  These  instruments  are  chiefly  x^sed  for 
the  measurement  of  the  grasp.  The  estimation  of  the  strength  of 
other  movements  is  practicable,  but  not  easy.  The  observer  opposes 
the  movement  by  means  of  the  instrument,  and  notes  the  resistance 
that  can  be  overcome.  Thus  pressure  applied  through  it  just  a^bove 
the  patella,  to  force  down  the  raised  thigh  in  the  sitting  posture,  shows 


10  SYMPTOMS. 

the  power  of  the  flexors  of  tlie  hip.  The  wide  variations  in  strength 
among  different  persons  renders  absolute  estimation  possible  only  when 
the  defect  is  considerable.  Slight  changes  are  recognised  by  comparing 
the  power  on  the  two  sides.  It  should  be  remembered  that  the  limbs 
on  the  right  side  are  normally  stronger  than  those  on  the  left,  the  rela- 
tion being  about  as  five  to  four.  Care  must  be  taken  to  secure 
uniformity  of  the  conditions  under  which  the  observations  are  made. 
A  careful  search  would  doubtless  reveal  average  proportions  between 
other  movements  that  can  be  measured.  Thus  the  strength  of  flexion 
of  the  hip,  estimated  in  the  way  just  mentioned,  often  corresponds  to 
the  strength  of  grasp  on  the  same  side. 

Inco-ordination  of  Movement. — By  this  term  is  meant  a  defect  in  the 
relative  time  and  degree  of  the  muscular  contractions  by  which  a  move- 
ment is  produced.  Every  movement  is  due  to  the  contraction  of  a 
series  of  fibres,  which  may  or  may  not  correspond  to  the  series  massed 
together  in  a  "  muscle."  Movements,  not  muscles,  are  represented  in 
the  structure  of  the  brain,  and  the  fibres  which,  together,  cause  a  simple 
movement  may  be  in  more  than  one  muscle,  or  may  be  only  part  of  a 
muscle.  The  synergic  action  of  the  several  fibres,  in  a  definite  degree 
and  at  a  certain  time,  is  essential  for  the  movement.  So  also  is  the 
simultaneous  contraction  of  the  opponents  of  these  acting  muscles, 
which  are  thus  steadied ;  this  antergic  *  contraction  is  essential  for  the 
exertion  of  force,  and  must  be  often  great  in  degree  when  strength 
rather  than  amount  of  movement  is  required.  It  must  also  be  lessened 
automatically  to  permit  movement.  (The  process  will  be  considered 
in  connection  with  the  functions  of  the  spinal  cord.)  Defect  in  the 
opponents  does  not  interfere  with  movement,  but  it  prevents  the  exertion 
of  force,  by  permitting  the  movements  to  be  too  great. 

It  is  not  customary  to  apply  the  term  "inco-ordination"  to  any 
derangement  of  movement  except  that  which  is  irregular  in  time. 
There  may  be  also  irregularity  in  degree,  but  it  is  when  the  contractions 
have  not  the  normal  duration,  or  are  not  synchronous,  or  not  in  due 
sequence,  that  the  conspicuous  irregularity  occurs  to  which  the  term  is 
applied.     This  is  also  often  called  "  ataxy." 

Its  manifestations  differ  in  character  according  to  the  character  of 
the  movement.  The  variations  are  so  great  that  it  is  not  practicable  to 
describe  them,  nor  is  it  necessary,  since  aU  have  the  general  nature  just 
stated.  It  is  also  not  easy  to  estimate  the  inco-ordination  so  as  to 
permit  definite  record.  When  the  movement  of  the  arm  is  affected,  the 
degree  may  be  estimated  by  making  the  patient  try  suddenly  to  touch, 
with  the  point  of  a  pencil,  a  spot  on  a  sheet  of  paper  placed  before 
him,  his  eyes  being  closed  at  the  moment  of  the  attempt.  After  a 
certain  number  of  attempts  the  average  distance  from  the  spot  of  the 
dots  he  has  made  is  ascertained,  and  this,  compared  with  the  result 

*  I  use  this  term  because  we  have  noiid  to  express  antagonistic  co-operation.  This 
■«  svnclironous  with  that  of  the  acting  muscle,  but  not  synergic.  ^ 


MOTOR.  11 

given  by  a  normal  individual,  affords  a  numerical  indication  of  the 
degree  of  in  co-ordination.*  The  method  is  effective,  although  the 
result  is  perhaps  scarcely  worth  the  trouble. 

Whenever  there  is  inco-ordination,  spontaneous  movements  occur  if 
the  patient  tries  to  hold  the  limb  in  a  certain  posture ;  the  variation  in 
muscTilar  contraction  prevents  jBxation  as  well  as  disorder  in  movement ; 
co-ordination  is  as  essential  for  steadiness  of  uniform  contraction  as  for 
the  steadiness  of  the  varying  contractions  of  movement.  These  involun- 
tary movements  constitute  a  delicate  test  for  the  presence  of  ataxy. 

All  spontaneous  muscular  contractions  derange  those  that  are  due  to 
the  will,  and  movement  is  disordered  by  any  "  tremor  "  which  co-exists. 
The  sHght  rapid  contractions  that  cause  "  tremor  "  may,  indeed,  occur 
only  as  voluntary  movement,  and  there  is  a  gradation  of  disorder 
from  simple  inco-ordination  to  that  which  may  be  called  "  tremulous." 
So  also  more  extensive  spasm  disturbs  movement  with  which  it  coincides, 
and  energetic  contractions  of  involuntary  nature  may  be  excited  by  a 
volitional  impulse.  Hence,  just  as  simple  ataxy  may  pass  into  tremor, 
so,  on  the  other  hand,  there  is  a  gradation  by  "  jerky  inco-ordination  " 
to  wide  sudden  movements,  the  violence  of  which  obscures  the  element 
of  mere  disorder. 

It  is  necessary  to  distinguish  co- ordination  and  the  direction  of 
movement  to  a  definite  end.  The  motor  process  for  the  latter  depends 
on  sensory  guidance,  chiefly  on  the  senses  of  touch  and  sight.  A  semp- 
stress with  loss  of  seDfa  ion  in  the  fingers  cannot  sew  unless  she 
constantly  watches  them.  A  compositor,  whose  sense  of  touch  is 
dulled  upon  the  tips  of  thumb  and  index  finger,  cannot  pick  up  the 
type,  or  if  he  does  pick  it  up  may  drop  it  again,  unless  he  constantly 
watches  the  movement.  There  is  also  a  guidance  from  the  afferent 
nerves  of  the  muscles,  but  this  is  related  to  co-ordination  proper  rather 
than  to  the  direction  of  movement,  and  it  wiU  be  more  conveniently 
considered  in  connection  with  the  functions  of  the  spinal  cord.  Indeed, 
the  mechanism  of  co-ordination,  and  its  special  factors,  can  only  be 
discussed  in  that  connection. 

Spasm. — Involuntary  muscular  contraction,  when  considerable  in 
degree,  termed  spasm,  is  always  due  to  the  influence  of  the  nervous 
system.  It  is  a  frequent  symptom  of  disease.  Three  chief  varieties 
may  be  distinguished,  but  iatermediate  and  combined  forms  are  often 
met  with.  (1)  We  may  have,  first,  a  single  isolated  very  brief  contraction 
of  one  or  more  muscles.  (2)  Separate  contractions  may  occur  in  rapid 
succession,  a  second  commencing  as  soon  as  there  is  relaxation  of  the 
muscle  from  the  first  contraction.  This  is  tenned  "  clonus,"  or  "  clonic 
spasm."  The  relaxation  of  one  is  often  incomplete  when  the  next  con- 
traction begins.  (3)  There  maybe  a  persistent  contraction  in  which  no 
relaxation  can  be  perceived.     This  is  tenned  "  tonic  spasm." 

(1)  It  will  be  i-emembered  that  muscles,  as  long  as  they  are  connected 

•  lihx,  '  Neur.  Centralb.,'  1884,  p.  83. 


12  SYMPTOMS. 

with  the  nerve-centres,  are  never  in  a  state  of  perfect  relaxation.  There 
is  always  a  certain  degree  of  persistent  slight  contraction  of  the  fibres, 
which  keeps  them  adapted  to  the  distance  between  thair  attachments, 
so  that  the  substance  of  the  muscle  presents  always  a  similar  degree  of 
firmness.  This  physiological  "  tone  "  is  due  to  a  continuous  action  of 
the  centre  on  the  muscles,  through  the  motor  nerves ;  but  this  action  is 
apparently  determined,  in  degree  at  least,  by  the  sensory  impressions 
from  the  parts,  and  especially  from  the  muscles.*  Tonic  spasm  may 
be  regarded  as  an  augmented  degree  of  this  state,  due  to  various 
causes.  Although  it  necessarily  depends  directly  on  the  action  of  the 
centres  in  the  spinal  cord,  this  may  be  due  to  the  augmenting  influence 
of  the  cerebral  centres,  or  to  diminution  of  the  latter,  permitting 
insubordinate  activity,  and  is  often  clearly  the  result  of  a  reflex  process. 
It  must  be  distinguished  from  structural  shortening  of  the  muscle. 
The  distinction  is  easy^  because  tonic  spasm  can  always  be  overcome  by 
gentle,  steady  traction,  while  structural  shortening  cannot  be  thus 
removed.  The  latter  may  result  from  long-continued  tonic  contraction ; 
nutritional  changes  alter  the  structure  of  the  muscle  and  fix  the 
elements,  espec-ally  the  interstitial  tissue,  in  the  state  of  contraction. 
It  occurs  whenever  muscles  for  a  long  time  are  unextended  by  their 
antagonists,  and  so  results  from  the  long-continued  maintenance  of  a 
certain  posture,  and  also  in  extreme  degree  from  paralysis  of  opponents. 
Its  influence  on  the  joints  and  the  parts  moved  causes  the  various  de- 
formities that  result  from  local  palsy.  The  tonic  spasmodic  shortening 
which  can  be  overcome  is  often  spoken  of  as  "  spastic."  It  is  some- 
times also  distinguished  as  "  contracture,"  the  term  "  contraction " 
(which  really  includes  all  forms  of  spasm)  being  then  apphed  in  a 
narrow  sense  to  the  fixed  shortening. 

(2)  Single  muscular  contractions  vary  much  in  extent  and  duration ; 
when  partial  they  sometimes,  as  already  stated,  seem  to  be  of  local 
origin.     When  more  extensive  they  depend  on  the  nerve-centres. 

(3)  Clonic  spasm  consists  of  a  series  of  brief  contractions,  each  of  which 
corresponds  to  that  which  is  caused  by  a  single  induction-shock  applied 
to  the  nerve.  This  has  a  definite  duration  of  one  tenth  to  one  twelfth  of 
a  second,  but  it  is  often  prolonged  by  the  force  which  the  contracting 
muscle  has  to  overcome.  In  the  serial  contractions  to  which  the  term 
•'  clonus  "  is  applied  there  is  also  an  interval  between  the  end  of  one 
and  the  commencement  of  another,  occupied  by  the  process  of  stimula- 
tion. The  '-foot-clonus"  to  be  presently  described,  which  consists  of 
a  series  of  such  contractions,  varies  in  frequency  from  five  to  eight  or 
ten  per  second.  The  contractions  in  "  tremor,"  as  seen  for  instance  in 
"  paralysis  agitans,"  have  a  somewhat  greater  average  frequency. 

•  Jendrassik  ('Neur.  Centralbl.,'  1896)  has  endeavoured  to  estiinate  tho  degree 
of  tone  by  the  resistance  of  the  muscles  to  passive  extension,  and  finds  it  greater  in 
spastic  paraplegia,  and  less  in  tabes  tbMU  iu  normal  states.  The  latter,  however,  pre- 
sented snch  wide  variations  as  to  preclude  absolute  inferences.  The  amount  of  the 
extending  force  applied  seems  to  need  more  careful  adjustment. 


SENSORY.  13 

Tetanic  spasm  is  produced  by  the  blending  of  clonic  contractions 
whicli  may  be  so  fused  that  the  contraction  is  uniform,  and  is  then 
identical  with  tonic  spasm ;  or  there  may  be  commencing  relaxation  of 
one  before  the  next  contraction  supervenes.  This  is  chiefly  seen  as 
such  spasm  develops  or  lessens.  .The  passage  of  tonic  into  clonic 
spasm  is  well  seen  in  the  severe  epileptic  convulsion,  in  which  the  fixed 
spasm  becomes  first  vibratory,  then  intermittent,  and  at  last  remittent. 

Sensory  Symptoms. — Altered  sensation  is  another  very  common 
symptom.  It  has  often  to  be  searched  for,  because  a  significant  defect 
may  be  unknown  to  the  patient.  Each  form  of  sensation,  touch, 
temperature,  and  pain,  must  be  separately  tested,  since  one  may  be 
affected  and  not  another.  The  affection  of  one  form  only  is  sometimes 
of  important  diagnostic  significance. 

Stnsibility  to  Touch.  — The  structures  that  subserve  tactile  sensibihty 
are  stimulated  chiefly  by  motion — by  change  in  contact.  Prolonged 
contact,  unif oiTQ  in  its  degree  and  place,  is  perceived  but  little,  and 
only  by  close  attention.  It  is  the  change  that  excites  a  nerve  impulse ; 
the  commencement  of  contact  is  more  effective  than  its  cessation, 
but  in  each  case  rapidity  of  change  chiefly  determines  the  result. 
a  firm  pressure  may  be  scarcely  noticed  if  produced  very  gradually, 
while  a  sudden  touch  is  observed,  however  light.  In  testing  tactile 
sensibility  care  must  be  taken  that  the  instrument  employed  does  not 
give  an  impression  of  heat  or  cold,  lest  the  patient  perceive  by  the 
sense  of  temperature  that  which  he  cannot  perceive  by  the  sense  of 
touch.  The  observer's  finger  may  be  used,  if  it  is  not  cold,  or  the  skin 
may  be  touched  with  a  feather,  a  piece  of  string,  of  India  rubber,  &c. 
The  eyes  of  the  patient  should  be  closed  during  the  examination,  and, 
since  he  is  apt  to  fancy  that  he  feels  a  light  touch  when  he  does  not,  he 
should  occasionally  be  asked  if  he  can  feel,  when  no  contact  is  made. 
But  it  is  easy  to  make  the  test  too  fine.  Where  the  skin  is  thick,  or 
sensibility  is  normally  low,  a  Hght  touch  with  a  piece  of  string  or  a 
feather  may  normally  be  unperceived.  Loss  of  sensibility  to  touch  is 
teinned  "  anaesthesia,"  but  this  word  is  often  applied  loosely  to  all  forms 
of  impaired  sensibility. 

In  examining  the  tactile  sensibility  it  is  important  to  ascertain  not 
only  whether  the  patient  can  feel,  but  whether  he  is  able  to  recognise 
the  place  touched, — ^whether  he  can  correctly  "  locahse  "  the  sensation. 
For  this  he  must  be  asked  not  only  whether  he  feels  the  touch,  but 
also  to  say  or  point  out  where  he  feels  it.  The  part  touched  should  be 
frequently  varied.  Another  test  for  tactile  sensibility  depends  on  the 
fact,  ascertained  by  E.  H.  Weber,  that  the  distance  apart  at  which 
two  points  are  discriminated  is  nearly  the  same  in  different  individuals 
in  the  same  part  of  the  body,  although  it  varies  much  in  different 
parts  of  the  body.  The  normal  distance  being  laiown,  the  increased 
distance  at  which  the  points  have  to  be  placed  for  their  discrimination 
is  a  measure  of  the  degree  of  defect.     The  points  should  not  be  so 


14  SYMPTOMS. 

sharp  as  to  occasion  pain.  Common  compasses  may  be  employed, 
but  the  most  convenient  "  aesthesiometer  "  is  one  contrived  by  Sieveking, 
in  which  the  points  are  attached  to  a  graduated  bar.  The  greater  the 
degree  of  tactile  sensibility,  the  nearer  together  can  the  points  be,  and 
still  be  discriminated.  The  distance  at  which  they  are  normally  distin- 
guished must  be  known  before  any  inference  can  be  drawn.  The  most 
important  average  distances,  ascertained  by  Weber,  are  as  follows ; — 
The  distance  is  smallest,  i.  e.  the  sensitiveness  is  greatest,  at  the  tip  of 
the  to  ague,  where  the  points  are  discriminated  when  only  1"5  mm. 
apart.  Then  come,  in  order  of  sensitiveness,  the  finger  tips,  2  to  3  mm. ; 
the  hps,  4  to  5  mm. ;  the  tip  of  the  nose,  6  mm. ;  the  cheeks  and  the 
backs  of  the  fingers,  about  12  mm. ;  the  forehead,  22  mm. ;  the  neck, 
34  mm. ;  the  forearm,  lower  leg,  and  back  of  foot,  40  mm. ;  the  chest, 
45  mm. ;  the  back,  60  mm. ;  the  upper  arm  and  thigh,  75  mm.  Slight 
variations  exist  in  different  individuals,  and  a  deviation  from  the 
normal  that  is  tmiform  in  degree  throughout  the  body  is  probably 
physiological.  In  using  this  test  it  is  necessary  to  touch  the  skin  with 
the  two  points  at  the  same  moment,  and  with  equal  pressure.  The 
examination  requires  time  and  patience,  and  the  results  are  considerably 
modified  by  the  intelligence  of  the  patient.  Moreover  the  power  of 
discrimination  is  increased  by  practice.  It  is  rare  to  obtain  a  con- 
clusive result  unless  there  is  a  degree  of  defect  that  causes  a  slight 
absolute  loss.  For  these  reasons  the  practical  value  of  the  test  is  less 
than  was  at  first  anticipated,  and  it  is  not  often  employed.  It  is  most 
useful  for  the  estimation  of  changes  of  sensibihty  in  the  same  person 
and  the  same  part. 

Curious  modifications  of  tactile  sensibility  are  sometimes  observed. 
In  one  of  these,  a  single  touch  is  felt  as  if  it  were  two  or  even  three,  a 
condition  that  has  been  termed  "  poljsesthesia  "  (Fischer).  In  another, 
an  impression  on  one  part  is  referred  to  some  other  part,  usually  in  the 
same  hmb,  rarely  in  another  limb.*  In  another  variety,  an  impression 
on  one  part  has  been  referred  to  the  corresponding  place  on  the 
opposite  side  of  the  body  ;  this  has  been  termed  "  allocheiria " 
(Obersteiner) . 

Perception  of  the  degree  of  pressure  on  the  skin  is  probably  sub- 
served by  the  nerves  for  tactile  sensibility.  It  may  be  estimated  by 
simple  pressure  apphed  through  any  instrument,  or  by  small  weights, 
or  by  an  instrument  contrived  for  measuring  the  tension  of  the  pulse 
in  which,  by  means  of  a  spring,  the  part  tested  must  be  supported,  so  that 
the  muscles  are  not  brought  into  action.     The  point  to  be  ascertained 

*  In  most  i)ersons  tliere  are  spots  in  the  skin  whence  a  painful  sensation  seems  to 
dart  to  a  distiint  place;  e.g.  a  prick  on  tlie  thigh  in  a  limited  area  may  be  felt  also 
as  a  sharp  pain  near  the  scapula.  This  shows  how  structural  connections  must  exist 
favouring  tlie  "reference"  of  sensation  to  a  distance  from  the  nerve  stimulated. 
Another  instance  of  tiie  same  perverted  reference  is  presented  by  the  frequent 
neuralgic  pain  in  one  jaw  due  to  a  carious  tooth  in  the  other  jaw,  which  seema 
painless. 


SENSORY.  15 

is  the  minimum  variation  that  can  be  recognised.  In  health  this  is 
about  one  twentieth  of  the  total  pressure,  whatever  the  latter  may  be 
(Weber).  The  sensation  in  the  skin  caused  by  electricity,  especially 
by  faradism,  does  not  always  correspond  to  that  due  to  other  stimuli. 
It  has  been  thought  to  depend  on  a  special  form  of  sensibility,  but 
the  evidence  of  this  is  insujGB.cient.  Its  features  and  their  meaning 
have  yet  to  be  ascertained. 

Sensibility  to  pain  is  subserved  by  what  are  called  nerves  of  "  common 
sensibihty."  It  may  be  tested  by  a  prick  or  a  pinch.  For  a  prick, 
too  fine  a  point  must  not  be  used,  not  only  because  a  sharp  point  may 
penetrate  the  skin,  but  because,  in  the  less  sensitive  parts  of  the  skin, 
where  the  nerve-endings  are  not  close  together,  a  fine  point  may 
be  unfelt  at  one  spot,  although  felt  readily  at  a  place  close  by. 
Nothing  answers  better  than  the  point  of  a  quill  pen.  The  faradic 
current  may  be  employed  to  test  sensibihty  to  pain,  wire  terminals 
being  most  suitable  for  the  purpose.  Its  advantage  is  the  delicacy 
with  which  it  can  be  graduated ;  but  it  does  not  furnish  any  absolute 
standard,  and  needs  more  study  before  it  can  be  usefully  employed. 

Sensibihty  to  pain  may  be  changed  with  or  without  sensibihty  to 
touch.  Its  loss  is  termed  "  analgesia,"  but  is  often  included  in  the 
general  term  "  anaesthesia."  A  painful  sensation  may  be  felt  more 
intensely  than  normal ;  this  is  usually  called  "  hyperaesthesia  " — a 
general  term  for  increased  sensitiveness  ;  sometimes,  with  more  pre- 
cision, "  hyperalgesia."  Occasionally  a  touch  on  the  skin  gives  rise 
to  pain,  but  it  is  probable  that  this  is  due  to  the  stimulation  of  the 
over-sensitive  nerves  of  common  sensibility,  and  is  not  an  intensification 
of  a  tactile  sensation.  The  fact  that  touch  may  be  perfect,  although  no 
paia  can  be  caused,  makes  it  difficult  to  conceive  that  the  nerves  of 
touch  can  subserve  pain.  Both  tactile  and  painful  impressions  may 
produce  sensations  that  are  abnormal  in  character,  described  as 
"  thriUing,"  "  tiaghng,"  &c.  This  perverted  sensation  has  been  termed 
"  paraesthesia,"  or  "  dyssesthesia,"  words  that  have  also  been  apphed 
to  purely  subjective  sensations. 

When  a  prick  is  perceived  as  touch,  pain  being  lost,  care  is  needed 
that  the  patient  shall  clearly  know  what  is  desired.  Since  there  is 
often  delay,  when  there  is  not  loss,  he  should  be  told  to  say  "  touch  " 
or  "  prick  "  the  instant  either  sensation  is  perceived.  When  sensibility 
to  pain  is  diminished  but  not  lost,  the  test  must  be  kept  uniform. 
Another  source  of  error  is  then  occasioned  by  the  readiness  with  which 
the  patient  may  mistake  the  degree  of  pain  he  is  required  to  recognise, 
and  by  the  fact  that  his  idea  often  varies  in  the  course  of  the  examina- 
tion. He  may  thus  deny  that  he  feels  a  prick  as  such  at  a  part  where 
presently  he  says  he  does,  and  this  quite  honestly,  the  sensation  being 
really  the  same.  It  is  probable,  indeed,  that  this  is  the  most  frequent 
cause  of  the  conclusion  that  the  state  "  varied  in  the  course  of  the 
examiiinfion,"  or  that  "the  patient's  answers  were  unreliable." 

Sensiotlity  to  temperature  is  usually  affected  with  sensibility  to  pain, 


16  SYMPTOMS. 

but  often  not  in  t!ie  same  degree,  and  one  may  be  impaired  withont  tlie 
other.  There  may  be  an  absolute  inability  to  recognise  either  heat  or 
cold  as  such,  or  slight  degrees  of  each  may  be  imperceived  while  con- 
siderable degrees  are  recognised  correctly.  In  the  latter  case  there  is 
impairment  of  the  power  of  perceiving  differences  in  temperature, 
analogous  to  the  defect  in  the  perception  of  differences  in  pressure. 
There  may  be  also  perverted  sensibility,  whereby  hot  objects  feel  cold, 
and  cold  objects  hot.  The  pain  which  extreme  degrees  of  temperature 
normally  produce  may  be  felt  with  undue  readiness,  or  less  readily 
than  normal.  When  the  interval  is  increased  that  elapses  before  a 
sensation  of  pain  is  felt,  there  is  a  similar  delay  in  the  sensation  of 
temperature.  It  must  be  remembered  that,  in  health,  this  sensation  is 
less  quickly  produced  than  that  of  pain,  because  time  is  required  to 
raise  the  temperature  of  the  sldn  sufl&ciently  to  enable  the  heat  to 
stimulate  its  nerves.  For  rough  examination,  hot  and  cold  spoons 
may  be  employed,  or  a  hinged  tongue  depressor,  one  half  of  which  is 
warmed,  or  two  similar  test-tubes,  one  containing  hot  and  the  other 
cold  water.  Both  heat  and  cold  must  be  sufficient  in  degree  to  be 
unquestionable,  so  that  the  doubt  of  a  patient  is  certainly  pathological, 
and  not  due  to  uncertain  "  lukewarmness."  For  ascertaining  the  power 
of  differential  discrimination,  large  test-tubes  may  contain  water  at 
known  temperatures,  indicated  by  a  thermometer  in  each. 

It  is  always  necessary  to  test  separately  the  sensibility  to  heat  and 
to  cold,  as  there  is  reason  to  beheve  that  they  are  subserved  by 
different  nerves.  If  very  minute  points  of  the  skin  are  examined,  it 
has  been  found  that  at  some  points  only  heat,  at  others  only  cold,  is 
perceived.  The  stimulation  of  points  of  the  skin  by  electricity  is  said 
also  to  show  that  at  some  points  the  peculiar  electrical  sensation  is 
produced,  at  others  a  sensation  of  cold,  at  others  one  of  heat.  Further, 
the  points  identified  as  "  cold  points  "  and  "warm  points  "  by  the  one 
method  are  said  to  correspond  with  those  ascertained  by  the  other.* 
The  conclusion  from  these  observations  is  confirmed  by  the  fact  that 
in  disease  the  sensibility  to  heat  and  to  cold  may  be  affected  in  different 
degrees.  Commonly,  however,  the  defect  in  the  two  corresponds; 
accuracy  in  the  comparison  is  beset  with  great  difficulty. 

Muscular  Sensibility. — The  term  "muscular  sense,"  as  often  used, 
includes  more  than  one  form  of  sensation.  There  is,  first,  a  true 
mascular  sensibility.  The  muscles  are  abundantly  supplied  with 
sensory  nerves,  which  end  in  the  interstitial  tissue  between  the 
muscular  fibres  in  special  structures,  fusiform  in  shape,  and  hence 
termed  "  muscle  spindles."  These  have  long  been  known,  but  have 
lately  been  investigated  by  Sherrington,  Batten,  and  others.  They 
are  of  considerable  length,  some  nearly  half  an  inch  long,  and  have  a 
special  relation  to  the  afferent  fibres,  which  seem  to  begin  in  them, 

*  Blix, '  Zeit-chr.  f .  Biolocrie,'  Bd.  xx,  p.  141 ;  Eulenberg,  *  Zeitschr,  f.  klin.  Med.,' 
Bd.  ix.  Heft  2.  The  observations  liave  also  been  corroborated  by  Goldscheider  and 
HL-rzen,  but  the  subject  deserves  further  investigation. 


SENSORY.  17 

although  the  strange  fact  that  they  contain  striated  muscular  fibres 
makes  it  probable  that  motor  fibrils  may  enter  them.  Their  relations, 
degenerative  and  morbid,  have  yet  to  be  worked  out,  but  we  may 
assume  that  it  is  probably  in  them  that  the  afferent  impulses  arise. 
Such  impulses  are  excited  especially  by  pressure,  and  by  traction  on  the 
muscle.  Thus  a  squeeze  of  the  calf  causes  a  muscular  sensation 
of  an  undeniable  quality,  and  strong  passive  flexion  of  the  foot  on 
the  leg  causes  a  sensation  which  is  distinctly  produced  in  the  muscle. 
The  nerves  are,  moreover,  strongly  stimulated  when  the  fibres  are 
widened  by  the  extreme  contraction  of  "oramp,"  which  causes 
acute  pain.  It  must  be  ascribed  to  the  compression  of  these  nerve- 
endings,  since  no  afferent  impressions  seem  to  come  from  within  the 
fibres.  It  is  thus  a  proof  of  their  susceptibihty  to  pressure-excita- 
tion. After  cramp,  moreover,  the  nerves  are  left  in  a  state  of  such 
exalted  excitability  that  even  slight  extension  of  the  muscle  gives  rise 
to  pain,  although  such  extension  has  no  sensory  effect  in  the  normal 
state.  Every  one  has  experienced  the  effect.  We  shall  afterwards  see 
the  importance  of  the  fact  that  the  nerves  are  chiefly  stimulated  by 
these  two  mechanical  processes. 

The  muscular  pain  caused  by  electrical  stimulation,  as  distinct  from 
the  pain  felt  in  the  skin,  may  be  due  to  the  stimulation  of  the  afferent 
nerves  in  excessive  degree,  or  to  the  effect  on  them  of  the  muscular 
contraction  that  is  produced;  the  former  is  the  more  probable.  But 
these  sensations  of  pain,  although  due  to  "muscular  sensibility,"  are 
not  included  in  the  term  "muscular  sense."  They  are,  however,  of 
great  importance  on  account  of  the  conclusive  evidence  they  afford  that 
afferent  impulses  come  from  muscles  and  as  to  the  way  in  which  they 
are  produced. 

The  chief  knowledge  that  is  ascribed  to  the  "muscular  sense"  is 
that  of  the  character  of  movement  and  posture  due  to  the  action  of 
the  muscles,  of  the  degree  of  movement  and  energy  of  contraction,  and 
also  of  the  character  of  passive  movement  (by  an  external  force)  and  of 
posture  when  the  muscles  are  at  rest.  It  is  evident,  however,  that  here 
we  have  two  very  different  conditions,  in  one  of  which  there  is  much 
activity  of  all  the  motor  elements,  nervous  and  muscular,  while  in  the 
other  these  are  almost  at  rest,  only  such  gentle  action  persisting  as 
maintains  the  "  tone."  Except  in  absolute  relaxation,  there  is  probably 
continuous  stimulation  of  the  afferent  nerves  by  this  tonic  state  of  the 
muscular  fibres.  In  active  contraction,  this  stimulation  is  far  greater, 
and  activity  of  the  motor  nerve-centres  is  added.  Hence  two  elements 
*re  comprehended  in  the  term,  and  must  be  separately  considered. 

Our  knowledge  of  active  states  of  the  muscle  is  due,  at  least  in  some 
measure,  to  the  effect  on  consciousness  of  the  activity  of  the  nerve- 
structures  causing  the  movement.  The  proof  of  this  consists  in  three 
facts.  (1)  In  palsy  of  an  ocular  muscle,  objects  seen  are  referred  to 
the  position  (in  relation  to  the  body)  that  they  would  occupy  if  the 
movement  corresponded  to  the  innervation ;  it  is  the  latter,  i.  e.  the 

VOL.  I.  2 


18  SYMPTOMS. 

activity  of  the  centre,  to  wliicli  the  perception  corresponds.  (2)  After 
amputation  of  a  limb,  a  person  who  makes  an  effort  to  move  the  lost 
part  seems  to  feel  as  if  he  did  move  it.  (3)  In  some  convulsions 
beginning  locally,  slight  attacks  may  be  attended  "with  a  feeling  that 
the  arm  is  raised  above  the  head,  or  otherwise  moved,  when  it  is 
hanging  by  the  side.  These  facts  show  that  the  central  motor  process 
is  an  important  source  of  our  knowledge.  But  to  its  effect  must  be 
added  that  of  the  impulses  from  the  muscles,  about  to  be  described. 

Our  knowledge  of  rest-posture  and  passive  movement  must  be 
derived  from  incoming  impulses.  These  are  not  from  the  skin ;  the 
sense  of  posture  may  be  lost  when  cutaneous  sensibihty  is  normal,  and 
perfect  when  this  is  much  impaired.  This  is  observed  in  tmilateral 
lesions  of  the  spinal  cord,  and  in  other  diseases.  The  chief  source  of 
these  perceptions  must  therefore  be  the  deeper  afferent  nerves,  those  of 
the  muscles  and  joints,  perhaps  chiefly  of  the  muscles. 

These  afferent  impulses  are  no  doubt  continuously  generated,  but  do 
not  influence  consciousness  as  definite  sensations.  They  certainly  in- 
fluence the  activity  of  the  motor  centres  of  the  spinal  cord,  and  there  is 
reason  to  think  that  they  pass,  in  part,  to  the  cerebellum,  and  act 
through  it  on  the  cerebral  cortex,  determining  and  assisting  in  regu- 
lating its  activity.  This  influence  on  the  motor  centres,  by  the  effect  it 
produces,  is  apparently  one  chief  cause  of  the  perceptions  of  these 
passive  states.  The  active  state  of  the  motor  centres  of  the  cortex, 
however  it  is  produced,  has  an  effect  on  consciousness  and  enters  into 
perceptions  though  no  sensation  results.  This  may  be  combined  either 
with  true  sensation  from  the  parts  concerned,  or  with  the  result  of  stiU 
slighter  impulses,  to  be  discerned  only  by  close  attention,  to  the  joints, 
skin,  &c.,  of  which  we  seem,  at  first,  to  be  altogether  unconscious. 
We  are  thus  led  to  refer  our  perceptions  of  passive  posture  in  part  to 
the  same  structures  as  give  rise  to  our  perceptions  of  active  posture. 
Our  knowledge  is  the  result  of  such  perception,  and  not  of  true  sensation 
proper ; — ^we  perceive  and  know  that  which  we  do  not  feel. 

Our  recognition  of  resistance  to  contraction  affords  one  of  the  most 
delicate  tests  for  "  muscular  sensibility."  It  is  ascertained  by  testing 
the  ability  to  detect  differences  of  weight,  i.  e.  variations  in  the 
resistance  to  contraction.  In  tliis  examination  the  patient's  eyes 
should  be  closed,  and  the  objects  used  should  be  of  uniform  size. 
Leather  balls  like  small  cricket  balls,  containing  various  weights  from 
two  drachms  to  two  pounds,  are  in  use  at  the  Queen  Square  Hospital 
for  this  purpose.  To  eliminate  as  far  as  possible  the  stimulation  of 
the  cutaneous  nerves,  we  may  (1)  reduce  it  to  a  minimum  area,  placing 
the  weights  in  a  bag  suspended  by  a  string  to  the  part  to  be  tested,  so 
that  only  a  small  area  of  the  skin  shall  be  pressed  upon ;  (2)  increase 
the  area  (Hitzig) ,  and  thus  diffuse  it  in  diminished  degree ;  (3)  increase 
the  weight,  and  therefore  the  pressure  in  both  area  and  degree,  so  that 
the  addition  to  be  discerned  shall  bear  only  a  small  proportion  to  the 
total  stimulation. 


REFLEX.  19 

Thus  we  may  test  muscular  sensibility: — (1)  by  the  power  of 
discriminating  weigbt.  This  is  greater  than  in  the  case  of  the  nerves 
of  the  skin,  since  a  difference  of  one  fortieth  of  tbe  total  weight  can  be 
recognised  under  normal  conditions ;  (2)  by  the  sensitiveness  of  the 
m.uscles  to  pressure  and  traction ;  (3)  by  their  sensitiveness  to 
electrical  stimulation.  It  is  difficult  to  test  accurately  the  electro- 
sensibility  unless  the  sensitiveness  of  the  skin  is  lost,  or  is  removed,  as 
by  the  injection  of  cocain. 

The  power  of  recognising  passive  posture  is  also  regarded  as  a 
test  for  muscular  sensibility,  but  it  may  mean  much  more,  afterwards 
to  be  considered.  If  cutaneous  sensibility  is  normal,  the  parts  handled 
must  be  grasped  firmly,  and  pressed  on  both  sides,  so  that  the  direction 
of  pressure  may  not  suggest  the  posture.  The  patient  should  be  asked 
to  indicate  the  sensation  by  putting  the  limb  of  the  other  side  into  the 
same  posture.  Several  observations  should  be  made  to  eliminate  the 
chance  of  accidental  error. 

Eeflex  Action. — Many  symptoms  of  disease  of  the  nervous  system 
are  due  to  derangement  of  the  various  reflex  actions.  These  are 
numerous,  and  we  can  now  only  consider  their  general  characters. 
Each  action  is  effected  through  afferent  sensory  nerves,  efferent  motor 
nerves,  and  a  system  of  nerve-cell-processes,  and  intervening  sub- 
stances,— the  "reflex  centre."  The  centre  is  usually  complex  and 
often  extensive,  and  in  it  are  paths  of  different  "resistance,"  deter- 
mining the  form  of  reflex  action  and  its  extent,  according  to  the 
source  and  intensity  of  the  sensory  impression.  The  reflex  centre 
is  between  the  roots  of  the  sensory  and  motor  nerves  concerned,  and 
with  them  it  constitutes  what  may  be  termed  a  "reflex  arc."  The 
sensory  impulse  may  not  only  excite  a  motor  process,  it  may  also  pass 
up  to  the  brain,  and  influence  consciousness  as  a  sensation.  It  is 
probable  that  the  two  effects  are  subserved  by  the  same  nerve-fibres,  so 
far  as  the  cutaneous  nerves  are  concerned,  but  some  fibres  from  the 
muscles  pass  up  the  cord  without  connection  with  the  spinal  centres. 
Further,  the  reflex  centre  of  the  spinal  cord  is  to  some  extent  under  the 
control  of  encephalic  centres — a  point  of  very  great  importance. 

Two  forms  of  reflex  action  must  be  distinguished.  The  first  is 
that  excited  chiefly  by  stimulation  of  the  nerves  of  the  skin ;  the  second 
is  produced  by  the  stimulation  of  deeper  nerves,  chiefly  those  of  the 
muscles,  in  the  manner  just  described.  This  form  is  caused,  among 
other  ways,  by  tapping  a  tendon,  and  therefore  was  at  first,  and  still  is 
sometimes,  called  "  tendon-reflex  action  " — an  undesirable  name. 

Cutaneous  Reflex  Action. — The  reflex  movements  obtained  from  the 
skin  are  excited  rather  by  a  gentle  stimulation,  as  a  touch,  than  by  a 
strong,  painful  impression.  They  consist  of  a  single  movement  in  most 
instances,  either  quick  or  slow,  but  often  complex,  and  extensive  in 
proportion  to  the  character  of  the  stimulation  effected,  the  nature  of 
the  central  mechanism,  and  the  state  of  the  centre.     Often  muscles  at 


20  SYMPTOMS. 

a  distance  are  influenced  by  a  powerful  stimulation,  either  "mtli  others 
or  alone.  A  strong  impression  may  cause  a  reflex  action  so  wide  as  to 
involve  most  of  the  muscles  of  the  body.  As  a  rule  a  painful  impres- 
sion causes  quick  flexion  of  a  limb,  such  as  to  withdraw  it  from  the 
cause  of  the  pain.  Thus  a  prick  near  the  knee  causes  a  reflex  flexion  of 
the  hip.  In  other  cases,  where  withdrawal  is  impossible,  a  protective 
movement  results,  as  in  the  case  of  the  eyeUds. 

The  sHghter  degrees  of  excitation  cause  a  contraction  in  the  muscles 
close  to  the  part  of  the  skin  that  is  stimulated,  the  impulse  passing,  in 
the  spinal  cord,  only  to  the  related  motor  centres.  This  limitation  of 
the  process  renders  them  important  in  diagnosis,  especially  when  lost 
on  one  side  (see  the  chapter  on  the  functions  of  the  spinal 
cord).  The  cutaneous  reflex  actions  may  be  excited  at  almost 
any  part  of  the  skin,  but  at  some  parts  they  are  very  definite  in  cha- 
racter, and  are  distinguished  by  special  names.  The  most  important 
are  the  "plantar  reflex,"  from  the  sole ;  the  "  gluteal  reflex,"  a  contrac- 
tion in  the  gluteus  when  the  sldn  over  the  muscle  is  stimulated  ;  the 
"  cremaster  reflex,"  a  retraction  of  the  testicle  on  stimulation  of  the  skin 
on  the  inner  part  of  the  thigh  ;  the  "  abdominal  reflex,"  in  the  muscles 
of  the  abdominal  wall  when  the  skin  over  the  side  of  the  abdomen  is 
stroked ;  the  upper  part  of  this  reflex  is  a  very  definite  contraction  at 
the  epigastrium,  and  has  been  termed  the  "  epigastric  reflex."  A 
series  of  reflex  actions  may  be  obtained  in  the  muscles  of  the  back, 
the  highest  being  in  the  muscles  of  the  scapula.  Others  have  been 
distinguished  and  named,  but  this  process  may  easily  be  carried  too 
far.  These  spinal  reflex  actions  vary  in  their  excitability  in  different 
individuals,  and  are  always  more  readily  produced  in  the  young  than 
in  the  old.     They  may  be  increased  or  abolished  by  disease. 

In  the  region  of  the  cranial  nerves  the  most  important  reflexes  are 
those  of  the  eye, — (1)  the  conjunctival  reflex  ;  (2)  the  contraction  of 
the  pupil  on  exposure  of  the  eye  to  light ;  (3)  dilation  on  stimidation 
of  the  skin  of  the  neck. 

Muscle-reflex  Action ;  "  Tendon-reflexes.** — The  second  group  of 
phenomena  which  depend  on  reflex  action  were  first  systematically 
studied  by  Erb  and  Westphal  more  than  twenty  years  ago.  Attention 
was  called  to  them  in  this  country  by  G-rainger  Stewart  and  Buzzard  in 
1878.  They  have  since  been  the  subject  of  a  vast  amount  of  study  and 
discussion.  All  occur  in  muscles  that  are  in  a  state  of  slight  tension, 
and  are  produced  by  a  sudden  increase  of  this  tension  or  by  some  other 
sudden  mechanical  influence,  but  voluntary  contraction  superadded  to 
passive  tension,  diminishes  or  prevents  them.  They  are  distinguished 
according  to  their  seat,  and  vary  in  distinctness,  conspicuousness,  and 
the  ease  with  which  they  are  produced  according  to  the  mechanical 
conditions  of  the  muscles  and  their  attachments. 

The  first  of  these  is  the  jerk  of  the  leg  which  occurs  when  the 
patellar  tendon  is  tapped.     It  has  been  called  the  "  knee- phenomenon" 


KEFLEX. 


21 


by  Westphal,  the  "  patellar  tendon-reflex  "  by  Erb,  the  "  knee-jerk  " 
by  myself.  To  obtain  the  jerk,  the  knee  must  be  flexed  so  that  the 
quadriceps  f  emoris  is  gently  extended.  If  then  the  patellar  tendon  is 
struck,  the  quadriceps  contracts,  and  the  lower  leg  is  jerked  forward 
if  free  to  move.  The  blow  is  given  over  the  space  above  the  tibia 
where  the  tendon  can  yield,  so  that  there  is  a  sudden  increase  in  the 
tension  of  the  muscle.  The  most  convenient  position  is  with  the  knee 
to  be  tested  flexed  nearly,  but  not  quite,  at  a  right  angle,  by  being 
placed  over  the  other  knee  as  the  person  sits  (Fig.  1).  But  if  the  leg 
to  be  tested  is  stout,  its  tension  in  this  position  may  be  too  great  to 


Fig.  1. — The  Knee  jkek. 
The  dotted  line  indicates  the  movement 
which  follows  the  blow  on  the  patellar 
tendon. 


Fig.  2. — The  Knee-jekk. 
ileihod  of  obtaining  it  when  it 
is  not  readily  pioduced  in  the 
ordinary  way. 


permit  of  any  movement.  In  such  case  the  observer  may  place  his  arm 
beneath  the  patient's  thigh,  just  above  the  knee,  and  rest  his  hand  on 
the  patient's  other  knee  (Fig.  2).  Muscular  relaxation  is  sometimes 
more  readily  obtained  when  the  legs  hang  vertically  ;  or  the  foot  may 
rest  on  the  floor,  and  the  contraction  be  felt  by  the  hand  placed  on  the 
muscle ;  the  effect  of  the  recoil  and  of  the  muscular  contraction  must 
then  be  carefully  distinguished.  If  the  bent  fingers  of  each  hand  are 
hooked  together,  the  hands  pulled  strongly,  and  the  eyes  closed,  the 
movement  is  increased  in  degree — "  reinforcement,"  it  has  been  termed.* 
It  is  essential  that  the  flexors  also  should  be  free  from  voluntary  con- 
traction. This  contraction  may  be  ascertained  by  feeling  the  hamstring 
tendons,  and  pressure  on  these  by  the  fingers  often  helps  to  secure  re- 
laxation. The  blow  may  be  given  by  the  side  of  the  hand,  a  percussion 
hammer  (Fig.  2),  or  a  stethoscope  with  an  india-rubber  edge  to  the 
ear-piece.  If  the  jerk  is  doubtful,  the  skin  shoxdd  be  bared. 
•  A  useful  device  suggested  by  Jendrassik. 


22 


SYMPTOMS. 


The  same  contraction  may  be  obtained  by  other  modes  of 
suddenly  increasing  the  traction  on  the  muscle,  especially  when  this 
excitabihty  is  abnormal  in  degree.  A  blow  on  the  tibia  may  be 
effective,  but  a  method  which  has  much  practical  convenience  is 
this.  When  the  patient  is  lying  with  the  muscles  relaxed,  the  patella 
is  displaced  downwards  by  a  finger  placed  across  its  upper  edge  ;  a  tap 
on  the  finger  in  the  direction  of  pressure,  so  as  suddenly  to  increase 
the  tension,  excites  the  contraction  of  the  muscle  whenever  there  is 
even  slight  increased  excitability.  If  the  tension  is  maintained  when 
the  excitability  is  much  greater  than  normal,  the  single  contraction  is 
immediately  succeeded  by  a  second,  and  this  by  a  third,  and  so  on — a 
series  of  qtdck  clonic  contractions,  or  "  clonus,"which  will  be  considered 
presently  in  more  detail.  It  may  continue  as  long  as  tension  is  kept 
up,  but  instantly  ceases  when  the  muscle  is  relaxed. 

Similar  contractions  can  be  obtained  by  similar  measures  in  many 
other  muscles.  That  in  the  muscles  of  the  calf,  which  causes  a  move- 
ment at  the  ankle-joint,  is  especially  important.  If  the  calf  muscles, 
which  are  connected  with  the  Achilles  tendon,  are  made  tense  by 
gently  pressing  up  the  foot,  and  this  tendon  is  tapped,  the  muscles 
contract,  causing  a  slight  extension  movement  of  the  foot ;  just  as  the 
muscles  of  the  thigh  contract  when  the  patellar  tendon  is  struck.  The 
tendon  is  unsupported  just  as  is  the  patellar  tendon,  and  so  yields 
before  the  blow  increasing  suddenly  the  tension  on  the  muscles.  A 
tap  on  the  side  of  the  tendon  is  equally  effective ;  but  if  the  tendon  is 
so  firmly  supported  on  the  other  side  that  it  cannot  yield,  a  tap  which 
before  caused  the  contraction  will  not  do  so.  This  is  evidence  that  it 
is  not  the  mere  percussion  of  the  tendon,  but  the  increased  tension, 
which  constitutes  the  stimulation.  In  cases  in  which  the  excitability  is 
excessive — just  as  sudden  tension  in  the  thigh  muscles  will  cause  a  con- 
traction, followed  by  others  in  a 
continuous  series — so,  in  such 
cases,  if  the  calf -muscles,  which 
extend  the  ankle-joint,  are 
suddenly  put  on  the  stretch  by 
pressing  the  hand  against  the 
sole  of  the  foot  (Fig.  3),  the 
first  contraction  is  followed  by 
another,  and  they  recur  as  long 
as  the  tension  is  maintained. 
This  series  of  contractions, 
the  "foot-clonus"  or  "ankle- 
clonus  "  (or  "  foot-phenome- 
non" — Westphal),  is  a  very 
important  symptom.  The 
movement  is  remarkably  uni- 
form under  the  same  conditions,  varying  from  six  to  nine  contractions 
per  second.     By  attaching  a  writing  point  to  the  foot,  and  making  it 


Fia.  3. — Method  op  eliciting  thb 

FOOT-CLOKUS. 


EliPLEX.  28 


trace  a  line  on  a  revolving  cylinder  covered  with  blackened  paper, 
tracings  may  be  obtained  (Fig.  4),  which  are  almost  as  regular  as 


Ftd.  4. — Tracing-  of  the  Foot-ci.ontts  in  Paeaplegia. 
(The  tracing  reads  from  right  to  left.) 

those  of  a  tuning-fork.*  The  clonus  in  the  extensors  of  the  knee  has 
the  same  time,  and  the  two  are  of  the  same  nature.  A  clonus  quite 
similar  may  sometimes  be  obtained  in  other  muscles — peronei,  flexor 
brevis  pollicis.  Similar  contractions  may  be  obtained  in  the  muscles 
of  the  arms.  Normally  those  most  distinct  are  (1)  in  the  biceps  and 
supinator  longus,  obtained  by  a  tap  at  the  wrist,  especially  on  the  radius, 
to  which  the  biceps  tendon  is  attached ;  and  (2)  in  the  triceps,  by  a  tap 
on  its  tendon  above  the  olecranon.  When  excessive,  and  there  is  persis- 
tent contraction  in  the  flexors  of  the  fingers,  a  similar  contraction  can  be 
obtained  in  them,  or  in  the  supinators,  and  even  a  clonus,  by  maintain- 
ing the  increased  tension.  With  the  increased  excitabihty  of  disease, 
a  contraction  can  easily  be  obtained  in  the  masseters  by  tapping  the 
chin  when  the  jaw  is  depressed,  and  a  clonus  may  occasionally  be 
produced  in  these  muscles,  and  even  in  the  trapezius. 

When  a  tendon  is  tapped,  and  its  muscle  contracts,  the  occurrence 
has  so  much  the  aspect  of  a  true  reflex  action  that  it  was  generally 
assumed  to  be  such,  the  stimulus  being  the  excitation  of  nerves  in  the 
tendon.  This  view  received  apparent  confii-mation  by  the  discovery  of 
certain  facts.  (1)  That  there  are  nerves  in  tendon.  (2)  That  these 
phenomena  depend  for  their  occurrence  on  the  integrity  of  the  reflex 
path  to,  through,  and  from  the  spinal  cord,  and  are  arrested  by  a 
lesion  in  this  path.  By  experiments  on  animals  (in  whom  similar 
contractions  may  be  obtained)  it  has  been  found  that  they  are  pre- 
vented by  division  of  the  nerves  to  the  muscles,  by  division  of  either 
the  anterior  or  posterior  roots  of  the  spinal  nerves,  or  by  destruction 
of  the  spinal  cord.f 

These  experimental  facts   are  abundantly  confirmed   by  those  of 

*  Other  illustrations  will  be  found  in  the  writer's  paper  ('  Med.-Chir.  Trans.,' 
1879),  "  The  so-called  Tendon-reflex  Phenomena."  Since  then  tracings  have  been 
published  by  Charcot  and  many  others. 

f  See  Tschirjew,  'Archiv  fur  Psychiatrie,*  Bd.  viii.  Heft  3,  1878,  and  manj 
subsequent  investigations,  among  which  the  most  important  are  those  of  Waller 
(•  Journ.  of  Phys.,'  1896)  and  Sherrington  ('  Proc.  Roy.  Soc.,'  1893).  They  do  not 
involve  practical  questions,  such  as  to  make  their  description  needful  here. 


24  SYMPTOMS. 

disease,  conspicuously  in  peripheral  neuritis.  They  unquestionaWy 
show  that  some  reflex  action  is  essential;  but  (as  Westphal  main- 
tained from  the  first)  they  do  not  prove  that  the  contractions 
depend  on  a  simple  reflex  action.  From  the  fact  that  the  contraction 
is  caused  by  a  tap  on  the  tendon,  the  conclusion  that  the  contraction 
■was  a  simple  reflex  effect  of  the  stimtdation  of  its  nerves  seemed 
obvious,  so  the  name  "  tendon-reflex "  was  given  by  Erb,  and  its 
simpHcity  and  correspondence  with  the  conspicuous  facts  made  it  at 
once  ciu-rent,  and  it  has  remained  so.  Yet  the  theory  it  embodied  is 
not  only  erroneous,  but  it  obscures  the  general  relations  of  these 
phenomena,  which  are  of  great  importance  for  the  comprehension  of 
the  symptoms  of  disease.  Hence  it  is  necessary  to  explain  the  evidence 
in  some  detail,  and  the  facts  show,  usefully,  that  that  which  is 
"obvious"  may,  as  the  origin  of  the  word  suggests,  be  a  cause  of 
stumbling. 

(1)  The  contractions  occur  equally  when  the  connections  of  the 
tendon  and  all  its  nerves  have  been  divided.  (2)  They  occur  only 
when  the  muscle  is  in  a  state  of  slight  passive  tension,  and  the  tap  on 
the  tendon  can  increase  this.  With  a  relaxed  muscle,  a  pinch  of  the 
tendon  produces  only  a  wide  reflex  action,  like  that  from  a  pinch  of 
the  skin;  indeed,  this  follows  a  pinch  that  can  be  felt  when  the 
muscle  is  tense.  Definite  stimulation  of  the  nerves  of  the  tendon  never 
acts  on  its  muscle  only.  (3)  Not  only  must  the  tap  increase  the 
tension,  but  (as  we  have  seen  when  these  phenomena  are  excessive) 
any  similar  sudden  increase  has  the  same  effect,  e.gr.  ataponthe  bone  to 
which  the  tendon  is  attached.  That  on  the  depressed  patella  (p.  22), 
has  the  same  effect  as  a  tap  on  the  tendon  below. 

When  there  is  extreme  excess,  the  readiness  with  which  a  tap  on  the 
bone  is  effective  has  led  to  the  idea  that  the  stimulus  is  from  the 
periosteiun,  but  the  tap  is  only  effective  when  it  can  act  mechanically 
on  the  muscle.  It  must  be  on  the  bone  to  which  the  muscle  is 
attached,  and  in  the  direction  to  increase  its  tension. 

The  evidence  is  conclusive  that  the  contractions  are  not  excited  by 
stimulation  of  the  nerves  of  the  tendon ;  the  stimulus  originates  in  the 
muscle,  the  tendon  being  only  a  means  by  which  that  stimulation  is 
produced.  If  so,  is  not  the  contraction  itself  a  reflex  effect  F  It  was 
suggested  by  two  of  the  earliest  investigators,  Tschirjew  and  Westphal, 
that  the  contraction  is  local  in  production,  due  to  the  influence  of  the 
stimulus  on  the  whole  of  the  muscular  fibres  when  they  are  rendered 
specially  excitable  by  the  reflex  influence  of  the  tension  acting  through 
the  spinal  cord. 

A  reflex  action  takes  a  certain  time,  which  is  needed  for  the  impulse 
to  travel  to  and  from  the  cord,  and  for  the  reflex  process  to  occur  ia 
the  centre.  According  to  received  physiological  data,  an  interval  of  at 
least  one  fifteenth  of  a  second  would  be  needed  for  the  knee-jerk  if  it 
were  a  reflex  process,  and  rather  more  for  the  movement  at  the  ankle. 
I  have  found  that  when  the  Achilles  tendon  or  the  front  of  the  leg  is 


EEFLEX.  25 

tapped,  tlie  resulting  contraction  occurs  in  about  "033  sec*  The 
interval  for  the  knee-jerk  has  been  found  by  most  to  be  about  •04 
or  '033  or  '022  sec.f  Gotch  found  it  to  be  "025  sec,  and  that  between 
the  tap  on  the  tendon  and  a  true  reflex  contraction  of  the  opposite 
quadriceps  to  be  '11  sec.J 

But  it  is  certain  that  some  reflex  process  is  concerned  in  the  pheno- 
mena. We  have  still  to  account  for  the  irritability  which  permits  the 
local  stimulus  to  cause  a  contraction.  This  irritability  is  developed  by 
passive  tension.  If  the  muscle  is  relaxed,  the  fibres  may  contract  if 
they  are  struck  directly,  just  as  do  the  fibres  of  a  separated  frog's 
muscle,  but  no  contraction  can  be  produced  by  striking  the  tendon. 
Hence  we  must  assume  that  the  tension  excites,  by  a  reflex  influence, 
a  state  of  irritability  to  local  mechanical  stimulation, — such  as  that  of 
a  tap  on  the  muscle,  on  its  tendon,  or  even  the  vibration  from  a  tap  on 
the  bone  or  on  adjacent  parts.  But  only  that  form  of  mechanical 
stimulation  is  effective  which  suddenly  increases  the  previous  tension. 
It  is  only  because  the  tap  on  the  tendon  does  this  so  readily  that  the 
tendon  is  the  means  by  which  the  contractions  are  most  easily  pro- 
duced, and  through  which  they  have  been  chiefly  studied  and  pre- 
maturely named.  If  the  tension  put  on  a  muscle  is  gentle  and  gradual, 
it  may  only  develop  the  irritability,  and  an  additional  local  stimulation 
is  necessary  to  produce  a  visible  contraction.  If,  however,  the  tension 
is  sudden  and  forcible,  it  not  only  develops  the  irritability,  but  pro- 
duces visible  contraction  in  the  muscle  thus  rendered  irritable — as  in 
setting  up  the  foot-clonus.  I  have  shown  that  the  relaxation  of  the 
muscle,  between  the  successive  contractions,  is  not  complete :  there 
is  persistent  residual  contraction,  i.  e.  a  tonic  contraction  on  which 
the  clonic  contractions  occur.  When  one  clonic  contraction  is  over,  the 
tension  continuing,  a  second  is  instantly  developed. 

The  production  of  the  clonus  seems  thus  to  depend  on  the  limited  dura- 
tion of  single  muscular  contractions  (such  as  are  obtained  by  a  single 
induction  shock§),  the  effect  of  sudden  tension  being  exerted  on  all 
the  fibres  of  a  muscle  at  the  same  time.  In  the  state  of  excitability 
produced  by  the  moderate  tension  its  effect  on  the  relaxing  fibres  is 

•  '  Med.-Chir.  Trans.,'  1879,  p.  292.  An  erroneous  distinction  between  the  nnture 
of  the  knee-jerk  and  foot-clonus  was  made  in  this  paper. 

t  -039  sec,  Burckhardt;  -032— -034  sec,  Tschirjew  ;  "04  sec.  Brissaud  ;  -03— -04 
sec,  Waller;  '03  sec,  Eulenberg.  Some  of  my  own  measureinunts  ('Med.-Chir. 
Trans.,'  1879,  p.  275)  gave  a  longer  interval  when  the  movement  of  the  foot  was 
taken  as  the  indication  of'  the  commencing  contraction.  "  Load "  will  increase 
greatly  the  period  of  latent  stimulation,  probably  by  causing  the  initial  contiaction 
to  expend  itself  on  the  elasticity  of  the  muscle.  The  measurements  given  above 
were  obtained  by  recording  the  commencing  contraction  of  the  muscle. 

J  'Journ.of  Phys.,'  1896,  vol.  xx,  p.  322.  In  the  rabbit  he  found  the  interval 
for  the  tendon-contraction  to  be  only  "005  sec,  and  compares  this  with  Tigerstedt's 
measurement  of  the  interval  when  the  nerve  is  stimulated  ('01  sec,  twice  as  great), 
and  concludes  that  the  contraction  can  only  be  direct. 

§  See  Fostei-'s  '  Physiology,'  1888,  pt.  i,  p.  78. 


26  SYMPTOMS. 

tliat  of  a  fresh  stimulus,  and  they  contract  again.  We  have  no  facts  to 
suggest  that  such  serial  contractions  could  each  be  produced  through 
a  reflex  mechanism. 

We  have  seen  that  the  afferent  nerves  of  muscles  are  especially 
excited  by  tension  (see  p.  17).  If  there  were  reason  to  regard  each 
contraction  as  reflex  we  should  have  to  regard  these  nerves  as  the  seat 
of  the  excitation.  The  facts  on  all  relations  are  better  explained,  and 
some  pathological  facts  are  only  explained,  by  the  assiunption  that  the 
tension  causes  by  reflex  action  this  irritability. 

It  seems,  therefore,  that  the  term  "tendon-reflex"  is  altogether 
inaccurate.  The  phenomena  are,  according  to  the  explanation  above 
given,  dependent  on  a  "  muscle-reflex  "  irritabihty,  which  has  nothing 
to  do  with  the  tendons.  If  each  contraction  is  reflex,  it  must  be 
generated  by  an  afferent  impulse  from  the  muscle-nerves,  not  from 
the  tendon-nerves.  This  fact  seems  conclusive  and  inevitable.  Hence 
if  we  wish  to  describe  them  by  a  general  term,  it  is  best  to  employ  one 
which  does  not  involve  any  special  theory  of  their  nature.  They  have 
been  termed  "  tendon-muscular  phenomena,"  but  the  intervention  of 
tendons  is  not  necessary  for  their  production ;  the  one  condition  which 
all  have  in  common  is  that  passive  tension  is  essential  for  their  occiir- 
rence,  and  1  have  suggested*  that  they  be  termed  m«/o^a.iic  contractions 
(rnrnos,  extended).  The  irritability  on  which  they  depend  is  due  to 
and  demonstrative  of  a  muscle-reflex  action  which  depends  on  the 
spinal  cord.  It  is  highly  probable,  as  Tschirjew  suggested,  that  the 
condition  on  which  the  myotatic  irritability  depends  is  identical  with 
muscular  "tone."  Since  the  experiments  of  Heidenhain  it  has  been 
generally  admitted  that  "tone"  depends  on  tension,  and  is  a  reflex 
process. t 

A  true  "tendon-reflex"  may  be  excited  by  pinching  the  tendon, 
but  this  is  a  start  of  the  whole  limb,  precisely  such  as  results  from  a 
pinch  of  the  skin.  It  is  an  instance  of  the  pain-reflex  described  above. 
The  importance  of  discarding  the  tendon-reflex  theory  (and  therefore 
the  name)  is  great,  for  it  prevents  the  comprehension  of  the  true  nature 
of  other  phenomena  of  great  importance.  Chloroform  first  increases 
and  then  abolishes  the  irritabihty;  in  ether  narcosis  (except  when 
extremely  deep)  it  is  increased :  J  asphyxia  has  a  similar  effect,  at  first 
increasing,  then  aboHshing,  while  oxygen  simply  augments  the  excita- 

*  '  Diasnosis  of  Diseases  of  the  Spinal  Cord,'  2nd  edit.,  1881,  p.  29. 

t  By  some  interesting  researches  recently  published,  Mommsen  has  reached  tbe 
same  condasion,  that  muscular  tone  is  dependent  on  a  muscle-reHex  action  excited 
by  tension  acting  on  the  sensory  muscle-nerves  ('  Virchow's  Archiv,'  Bd.  ex,  p.  22). 
The  view  given  above  has  been  reremly  advocated  by  Gotch  as  the  result  of  his 
experiments  ('  .louni,  of  Physiology,'  1896),  and  is  indeed  now  generally  accepted 
by  physioliigists. 

X  'Diagnosis  of  Diseases  cf  the  S|iin;il  Cord,'  1883,  p.  31;  observations  hj 
Horsley. 


REFIEX.  27 

bility.*     Tlie  relation  of  these  contractions  to  the  nerve-centres  will 
be  considered  in  connection  with  these. 

Paradoxical  Muscular  Contraction. — This  name  has  been  given  by 
Westphal  t  to  a  slow  tonic  contraction  occurring  in  a  miascle  when  its 
attachments  are  suddenly  brought  nearer  in  some  morbid  states.  It 
is  best  seen  in  the  tibialis  anticus ;  if  the  foot  is  grasped,  and  passively 
flexed  on  the  leg,  the  tibialis  anticus  contracts,  sometimes  after  an 
appreciable  interval ;  its  tendon  stands  out,  and  the  contraction  keeps 
the  foot  flexed  for  a  time,  which  may  be  some  minutes  (in  one  case  for 
twenty-seven)  :  the  relaxation  is  slow.  Repetition  lessens  the  con- 
traction. The  contraction  is  not  voluntary  (although  a  voluntary 
contraction  may  simulate  it).  It  may  occur  on  one  side  only.  The 
contraction  produced  by  brief  faradism  may  be  similarly  prolonged  in 
the  same  case.  A  similar  contraction  may  be  observed  in  the  extensors 
of  the  toes,  sometimes  in  the  flexors  of  the  knee,  rarely  in  the  arm 
muscles.  It  seems  to  be  an  excessive  manifestation  of  a  physiological 
relation  by  which  suddenly  diminished  tension  increases  as  increased 
tension  lessens  the  activity  of  the  motor  centres.  Tension  stimulates  the 
afferent  nerves,  and  the  relaxation  of  the  opponents  of  a  contracting 
muscle  during  movement  seems  to  be  thus  produced,  as  is  made 
probable  by  the  absence  of  descent  of  the  upper  lid  in  palsy  of  the 
inferior  rectus. J  Sherrington  has  shown  that  if  the  nerve  to  a  muscle 
is  cut  and  its  central  end  stimulated  the  opponents  relax. §  Con- 
versely we  can  understand  that  diminished  tension  may  induce  over- 
action.  It  seems  to  be  an  excess  of  the  contraction  by  which  a  muscle 
adapts  itseK  to  passive  shortening  of  its  course.  Jn  the  cases  that  have 
presented  this  phenomenon  there  has  been  no  muscular  rigidity,  and 
no  great  excess  (even  sometimes  a  loss)  of  the  myotatic  irritability. 
It  may  occur  in  the  early  stage  of  tabes,  and  in  some  other  affections, 
but  its  &iignificance  is  not  known.  A  phenomenon  somewhat  similar 
occurs  in  hysteria  (Fere)  ;  it  seems,  indeed,  but  an  isolated  and  imper- 
fect instance  of  the  condition  that,  when  universal  and  perfect,  consti- 
tutes the  flexihilitas  cerea  of  catalepsy,  i.  e.  an  unrestrained  spinal  reflex 
process  in  the  centres  concerned  in  the  states  of  the  muscles. 

Changes  in  ISTtjtbitign. — The  nutrition  of  all  the  tissue  elements  is 
largely  under  the  influence  of  the  nervous  system.  Whether  this 
influence  is  exerted  through  special  "trophic"  nerves,  or  through 
the  motor,  the  sensory,  and  especially  through  the  vaso-motor  nerves, 
is  a  question  that  has  been  much  discussed  in  the  past.  The  balance 
of  evidence  is  against  the  existence  of  special  trophic  nerves  or  centres. 
The  fact  regarding  these  changes  that  is  clearest  and  most  important  is 

•  Risien  Russell  ('  Proc.  Roy.  Soc.,'  vol.  liii,  p.  430). 

t  'Arch.  f.  Psych.,'  Bd.  x,  p.  243. 

X  Sco  the  Author,  *  Med.-Chir.  Trans./  1879. 

§  'Brit.  Med.  Journ.,'  1893. 


28  SYiirxoMS. 

tliat  acute  disturb  ince  of  nutrition  is  the  result  of  irritative  changes 
in  the  nutrition  of  the  nerves,  and  is  in  proportion  to  the  intensity 
of  that  irritation.  If  a  thread  is  passed  through  each  sciatic  nerve, 
and  one  is  also  irritated  by  the  application,  from  time  to  time,  of  irritant 
liquids,  trophic  changes  occur  in  that  limb  with  greater  rapidity  and 
far  greater  intensity  than  in  the  other  (Lewaschew). 

The  same  fact  is  frequently  indicated  by  the  extreme  readiness  with 
which  trophic  lesions  of  the  skin,  and  even  cellular  tissue,  occur  in 
certain  forms  of  inflammation  of  the  spinal  cord,  especially  in  those 
that  have  a  strong  tendency  to  spread  in  the  cord,  and  to  the  nerves, 
if  they  reach  the  centres  of  them.  The  only  apparent  explanation 
of  the  various  facts  is  that  the  nerve-endings  are  related  to  the 
tissues  in  such  a  way  that  the  nutrition  of  the  molecules  of  the  tissues 
is  determined  in  its  character  by  that  of  the  nerves.  The  process  of 
irritation  descends  the  nerves ;  it  passes  from  them  to  the  tissues,  and 
extends  to  all  that  are  in  continuity.  Thus  we  can  understand  equally 
the  acute  changes  in  the  muscles,  which  will  be  described  as  resulting 
from  disease  of  the  motor  nerves,  and  also  those  in  the  skin  and  other 
tissues  that  are  under  the  influence  of  the  sensory  fibres. 

Nutritive  changes  in  the  skin  are  easily  recognised,  but  they  diffei 
much  in  their  character  according  to  their  acuteness.  When  they  are 
rapid,  and  due  to  very  acute  irritation  of  the  nerves  or  the  spinal  cord, 
such  as  those  just  mentioned,  the  temperature  of  the  limb  is  raised, 
the  vessels  readily  dilate,  and  remain  dilated  for  a  long  time,  bullae 
form,  containing  a  dark-coloured  liquid,  and  slight  pressure  occasions  a 
slough.  These  changes  sometimes  seem  to  occur  spontaneously,  but  are 
more  often  excited  by  some  cutaneous  irritation,  and  very  slight  irri- 
tation of  the  skin  will  suffice  to  produce  them.  Trifling  pressure  will 
set  up  a  slough,  and  extensive  vesication  may  result  from  the  applica- 
tion of  a  hot-water  bottle  that  is  not  more  than  pleasantly  warm  to  a 
healthy  hand.  When  the  nerve  irritation  is  intense,  effusion  may 
occur  into  the  joints. 

In  the  case  of  the  fifth  nerve,  acute  trophic  changes  occur  in  the 
eyeball,  chiefly  when  the  disease  involves  the  Gasserian  ganglion,  or 
the  nerve  in  front  of  it.  To  produce  the  same  effect,  a  lesion  behind 
the  ganglion  has  to  be  more  irritating  than  one  in  front  of  it,  and 
a  similar  relation  obtains  in  the  case  of  the  spinal  nerves  and  the 
spinal  gangha.  The  cells  of  the  ganglia  govern  the  nutrition  or 
their  processes  in  each  direction,  i.  e.  of  the  whole  neuron,  and  we  can 
understand  that  they  have  some  power  of  hindering  the  propagation 
of  in-itative  changes,  while,  if  these  invade  the  ganglion,  they  pass 
thence  with  special  energy.  Very  intense  changes  in  nutrition 
may  thus  pass  to  the  skin  in  acute  inflammation  of  the  spinal  cord. 

The  alterations  in  nutrition  in  chronic  lesions  (which  have  been 
carefully  studied  by  Paget  and  Weir  Mitchell)  differ  considerably  from 
those  that  result  from  acute  irritation.  There  is  a  slow  change  in  the 
nutrition  of  the  skin,  which  becomes  red,  thin,  and  shiny — the  "  glossy 


EEFLEX.  29 

skin"  of  Paget.  The  subcutaneous  tissue  also  wastes,  so  that  the 
finger  tips  become  pointed.  The  growth  of  the  hair  and  nails  is 
altered,  and  the  latter  become  brittle.  The  bones  may  suffer  in  their 
nutrition,  and  may  break  more  easily  than  in  health.  If  the  lesion 
occurs  during  the  period  of  growth,  this  is  retarded.*  These  changes 
seem  to  depend  on  slower  change  in  the  nutrition  and  vitality  of  the 
tissue  elements  corresponding  to  a  slower  change  in  that  of  the  nerves, 
an  atrophy  rather  than  a  degeneration,  f 

The  muscles  suffer  from  lesions  of  the  motor  nerves ;  the  minute 
changes  are  described  with  the  latter.  At  first  there  may  be  merely 
fiabbiness,  and  an  appearance  of  wasting  that  is  not  confirmed  by 
measurement,  but  there  is  soon  an  actual  diminution  in  the  circum- 
ference of  the  limb.  In  extreme  cases  all  the  muscular  tissue  dis- 
appears, and  the  contour  of  the  limb  is  considerably  changed.  In 
comparing  the  size  of  the  Hmbs  on  the  two  sides  it  should  be 
remembered  that  the  limbs  on  the  right  side  are  normally  some- 
what larger  than  those  on  the  left  side,  and  that  the  difference 
varies  according  to  the  occupation  of  the  individual,  and  the  degree 
in  which  this  involves  a  greater  use  of  the  limbs  on  one  side. 
In  measuring,  great  care  is  necessary  to  secure,  as  nearly  as  pos- 
sible, the  same  conditions  on  each  side,  both  as  regards  the  state  of 
the  muscles  and  the  place  of  measurement.  It  is  best,  wherever 
j'ossible,  to  take  the  maximum  measurement  in  each  part,  rather  than 
to  attempt  to  make  the  measurement  in  the  same  place.  In  the  calf 
the  maximum  circumference  should  always  be  taken.  In  the  thigh 
accurate  measurement  is  extremely  difficult,  because  a  maximum 
cannot  be  taken.  We  may  endeavour  to  measure  at  the  same  point 
in  the  thigh  at  the  same  distance  from  the  condyles,  but  it  is  very  diffi- 
cult to  be  exact,  and  a  more  accurate  comparison  can  often  be  mada 
by  taking  the  minimum  circumference  above  the  knee,  in  spite  of  the 
fact  that  the  muscular  tissue  there  is  small,  and  the  difference  less  than 
it  is  elsewhere.  In  the  forearm  the  best  result  is  obtained  by  taking 
the  maximum  measurement  around  the  muscular  prominence  below 
the  elbow,  over  the  supinator  longus.  In  the  upper  arm,  the  circum- 
ference is  nearly  the  same  in  the  middle  third,  and  the  measure- 
ment may  be  made  halfway  with  little  risk  of  error.  In  all  cases  care 
must  be  taken  to  draw  the  tape  equally  tight  at  each  place.  It  is 
easier  to  do  this  with  a  flexible  steel  measure  than  with  an  ordinary 
tape  measure.  Exactness  is  best  secured  by  having  a  spring  at  one 
extremity  of  the  tape  with  an  index  that  will  show  the  tension. 

Electrical  Irritability. — The  nerves  and  muscles  are  excitable 
by  electricity,  and  the  excitability  is  changed  by  disease,  of  which  the 

*  But  the  subject  of  hindrance  to  growth  is  complex,  and  still  not  clear.  See 
the  account  of  infantile  paralysis. 

t  Cf.  Maiinesco  on  degeneration  and  atrophy  ("La  Theorie  des  Neurones,** 
•  Presse  Med.,'  1895). 


30  SYMPTOMS. 

change  is  often  an  important  symptom.  It  indicates  the  state  oi 
nutrition  of  the  nerve-fibres  and  muscles,  and  from  this  we  can  often 
draw  important  inferences  regarding  the  condition  of  the  centres. 

In  the  normal  state  nerve-fibres  are  stimulated  by  either  the  induced 
or  the  voltaic  current,  the  stimulation  of  the  motor  nerves  being  shown 
by  contraction  in  the  muscles  supplied  by  them,  that  of  the  sensory 
nerves  by  the  sensation  that  is  caused.  The  contraction  of  the 
muscles  is  continuous  when  the  faradic  current  is  applied,  but  if  the 
isolated  shocks  of  which  the  current  consists  are  separately  passed, 
each  causes  a  brief,  momentary  contraction.  When  the  voltaic  current 
is  applied,  contraction  occurs,  with  a  current  of  moderate  strength, 
only  when  the  strength  of  the  current  is  changed,  and  chiefly  when  the 
current  commences  or  ceases  to  pass,  i.  e.  when  the  circuit  is  "made" 
or  "broken."  The  stimulation  of  the  sensory  nerves  is  greatest  at 
those  times,  but  occurs  also,  in  a  slighter  degree,  during  the  whole 
time  that  the  voltaic  cui-rent  is  passing.  Hence  this  is  probably 
the  case  also  in  the  motor  nerves,  although  the  stimulation  is  too 
feeble  to  produce  a  contraction  in  health  with  a  strength  of  current 
that  can  be  borne.  In  proportion  as  the  nutrition  of  the  nerve-fibres 
is  impaired,  their  excitability  is  lowered,  and  a  stronger  current  of  each 
kind  is  required  to  excite  them  and  cause  contraction  in  the  muscles 
they  supply.  When  their  nutrition  is  much  impaired — i.  e.  when  the 
fibres  are  "degenerated" — no  contraction  can  be  obtained  even  with 
the  strongest  currents. 

The  changes  in  the  excitability  of  the  muscles  are  less  simple,  because 
in  them  there  are  two  excitable  structures — ^the  terminal  branches  of 
the  nerves,  and  the  muscular  fibres  themselves.  Of  these  the  nerve- 
fibres  are  the  more  sensitive  to  faradism,  and  the  faradic  stimula- 
tion of  a  muscle,  under  normal  circumstances,  is  by  means  of  these 
motor  nerve-endings.  Thus  we  find  that  its  excitability  corresponds  in 
degree  to  that  of  the  motor  nerve  supplying  it.  The  muscular  fibres 
themselves  are,  even  in  the  normal  state,  less  sensitive  to  faradism 
than  the  nerve,  apparently  because  they  are  incapable  of  ready  response 
to  a  stimulus  so  very  short  in  duration  as  are  the  shocks  of  which  the 
faradic  "  current "  consists.  The  proof  of  this  consists  in  the  fact  that 
under  the  influence  of  curara,  which  removes  the  excitability  of  the 
terminations  of  the  motor  nerves,  the  muscle  requkes  a  stronger  faradic 
cm-rent  to  stimulate  it  than  in  the  normal  state.  But  under  the 
influence  of  curara,  or  when  the  nerve  is  degenerated,  the  slowly  inter- 
rupted voltaic  current  stimulates  the  muscle  as  readily  as  in  the  normal 
state  ;  a  contraction  occurs  when  the  circuit  is  completed  or  broken — 
slower  than  that  which  occurs  when  the  nerve-fibres  are  intact,  and 
due  to  the  stimulation  of  the  protoplasm  of  the  muscular  fibres  them- 
selves. The  fact  that,  under  normal  circumstances,  the  contraction 
which  is  caused  by  the  voltaic  current  is  as  quick  as  that  produced  by 
the  faradic  shock,  is  ground  for  believing  that,  in  health,  the  voltaic  as 
well  as  the  faradic  current  causes  the  muscle  to  contract  by  exciting 


REFLEX.  81 

the  motor  nerve-endings.  When  the  motor  nerve  is  degenerated,  and 
will  not  respond  to  faradic  or  voltaic  stimulation,  the  application  of 
the  former  to  the  xauscle  ceases  to  cause  contraction.  Apparently,  the 
nerve-degeneration  is  accompanied  by  changes  in  the  nutrition  of  the 
muscular  fibre,  by  which  any  power  of  response  to  faradism,  which  it 
possessed  in  the  normal  state,  is  lost.  But  not  only  does  the  response 
to  the  voltaic  current  remain ;  it  becomes  more  ready  than  in  health, 
doubtless  in  consequence  of  nutritive  changes  just  mentioned.  More- 
over there  may  often  be  observed  a  change  in  the  readiness  of  response 
to  the  two  poles  of  the  voltaic  current — a  "qualitative  "  change,  as  it 
is  termed.  In  health,  the  first  contraction  to  occur,  on  gradually 
increasing  the  strength  of  the  current,  is  at  the  negative  pole  when 
the  circuit  is  closed,  and  a  stronger  current  is  required  before  closure- 
contraction  occurs  at  the  positive  pole.  But,  in  the  morbid  state  we 
are  considering,  closure-contraction  may  occur  at  the  positive  pole  as 
readily  as  at  the  negative,  or  even  more  readily, — and  contractions, 
when  the  circuit  is  broken,  occur  far  more  readily  than  in  the  normal 
state.  This  condition,  then — ^faradic  irritability  lost,  voltaic  irritability 
increased  and  often  changed  in  quality — is  termed  the  "  degenerative 
reaction,"  because  it  occurs  when  the  nerve-fibres  are  degenerated ;  if 
we  test  them  we  shall  find  no  response  to  any  stimulus,  voltaic  or 
faradic.  It  thus  indicates  loss  of  excitability  in  the  motor  nerves 
within  the  muscles,  or  a  change  in  their  endings  on  the  fibres,  by 
which  these  cannot  be  excited,  either  by  electricity  or  by  nerve-impulses, 
in  the  fibres.  It  occurs  when  the  latter  has  been  produced  by  some 
toxic  agent,  or  the  fibres  also  are  degenerated  in  consequence  of  their 
damage  between  the  muscle  and  the  ganglion  cells  of  the  cord  of  which 
they  are  part,  or  destruction  of  the  cells. 

But  the  motor  nerve-celis  and  fibres  often  undergo  changes  in 
nutrition  of  a  much  more  chronic  character.  In  this  condition  the 
excitability  of  the  fibres  is  lessened  gradually  and  slowly.  The  irrita- 
bility of  the  intra-muscular  nerve-endings  is  lowered  in  the  same  degree 
as  that  of  the  nerve-trunks,  and  we  have  a  similar  diminution  to  both 
faradism  and  voltaism.  The  nutrition  of  the  musciJar  fibres  is 
slowly,  gradually  impaired;  and  when  the  nerve-fibres  are  much 
affected  the  muscular  fibres  are  also  There  is  no  stage  in  which 
the  nerve-fibre  excitability  is  lost,  and  the  muscle-fibre  excitability 
retained ;  hence  there  is  no  condition  of  lost  faradic  and  increased 
voltaic  excitability  such  as  characterises  the  degenerative  reaction 
just  described.  Excitability  is  changed  to  the  one  form  of  stimulus 
just  as  to  the  other. 

Between  these  two  forms  there  are  intermedia.te  conditions.  For 
instance,  the  nerves  may  present  normal  irritability,  while  in  the 
muscle  there  is  often  increased  voltaic  excitability  and  a  changed 
order  of  polar  reaction.  In  these  cases  some  nerve-fibres  are  degene- 
rated, and  lead  to  the  increased  excitability  of  some  muscular  fibres. 
In  both  nerve  and  muscle  the  character  of  the  reaction  is  manifested 


32  SYMPTOMS. 

by  the  more  excitable  structures  ;  hence  it  is  normal  in  the  nerve  and 
altered  in  the  muscle — this  has  been  termed  by  Erb  the  "  middle  form 
of  degenerative  reaction,"  It  is  more  accurate  to  call  it  the  "  mixed 
form." 

Another  common  intermediate  form  is  due  to  the  degeneration  of 
the  nerves,  although  slow,  being  less  slow  than  in  the  condition 
described  above.  Hence  the  nutrition  of  the  muscular  tissue  and  its 
excitability  (by  voltaism)  persist  longer  than  those  of  the  nerves,  and 
although  lowered,  are  diminished  less  than  the  faradic  excitability  of 
the  nerves  at  each  stage.  When  the  latter  is  lost,  voltaism  will  still 
cause  a  slight  contraction  in  the  muscles,  which  may  occur  first  at  the 
anode  instead  of  the  cathode.  Every  gradation,  moreover,  is  met  with 
between  this  and  the  true  reaction  of  degeneration.  This  will  be 
intelligible  on  consideration.  It  depends  chiefly  on  the  rate  at  which 
the  nerves  degenerate. 

The  lowered  excitability  of  the  nerves,  due  to  degeneration,  is  often 
preceded  by  a  slight  increase  of  irritability,  very  transient  when  the 
degeneration  is  acute,  of  longer  duration  when  the  degeneration  is  of 
the  slower  variety  j  ust  noticed.  In  some  morbid  states,  again,  in  which 
the  change  of  nutrition  in  the  cells  and  fibres  is  extremely  shght,  a.n 
increase  may  alone  be  discovered.  I  have  found  such  an  increase,  for 
instance,  in  diseases  regarded  as  functional,  as  paralysis  agitans  and 
chorea,  and  it  is  an  interesting  proof  of  the  molecular  changes  which 
underlie,  or  result  from,  so-called  "  functional  "  maladies. 

The  various  changes  in  irritability  were  formerly  thought  to  indicate 
the  existence,  and  various  affection,  of  separate  centres  for  the  nutri- 
tion of  the  nerves  and  muscles,  apart  from,  though  acting  through,  the 
motor  nerve-cells.  Remembering  that  the  fibres  of  the  nerves  and 
muscles  suffer  in  different  degrees,  as  above  described,  the  phenomena 
may  all  be  explained  on  the  simpler  principle  stated,  without  the 
assumption  of  these  special  centres,  of  the  existence  of  which  there  is, 
indeed,  no  evidence.  Special  trophic  nerves  and  centres  for  muscular 
nutrition  have  quietly  disappeared  from  physiology,  and  are  now  only 
matters  of  interest  to  the  student  of  its  history,  and  to  those  whose 
memories  reach  further  than  two  decades. 

It  is,  however,  important  to  remember  that  such  alterations  in 
nutrition  and  excitability  as  occur  in  the  whole  course  of  the  motor 
fibres  when  the  cells  are  affected  (of  which  they  are  the  prolonged 
processes)  may  have  a  different  origin  at  the  periphery.  They  are 
the  same  in  "degenerative  peripheral  neuritis,"  which  begins  in  the 
extremities  of  the  fibres  (where  vital  resistance  is  least  in  consequence 
of  distance  from  the  cell  body),  and  extend  for  a  variable  distance 
upwards.  But  this  condition  is  seldom,  if  ever,  so  slow  as  to  permit 
the  equal  loss  of  nerve  and  muscle  excitability  met  with  in  the  central 
degenerations. 


MUSCLES   OF   UPPER   LIMB.  88 

THE  MUSGLE8:    THEIE  ACTION  AND 
PABALY8I8, 

Disease  of  the  motor  nervous  system  is  largely  manifested  by  loss 
of  muscular  action.  Individual  muscles,  as  well  as  groups  of  muscles, 
are  often  separately  affected.  Hence  it  is  desirable  to  consider  the 
symptoms  of  the  paralysis  of  the  more  important  muscles,  before  we 
enter  on  the  study  of  special  diseases. 

The  symptom  of  the  palsy  of  any  muscle  is  a  loss  of  its  normal 
action,  and  a  knowledge  of  this  action  is  essential  for  the  comprehen- 
sion of  those  symptoms.  The  two  must  therefore  be  considered 
together.  They  are  positive  and  negative  aspects  of  the  same  facts. 
It  may  be  well,  at  the  same  time,  to  mention  the  nerve  by  which  each 
muscle  is  supplied,  and  also  the  spinal  roots  from  which  the  fibres 
come.  These  cannot  be  ascertained  by  dissection.  Of  the  several 
roots  that  join  to  form  the  cord  of  the  plexus  from  which  the  nerves 
to  two  muscles  come,  fibres  from  all  may  pass  iato  one  nerve,  and 
from  only  one  or  two  of  the  roots  into  another.  The  facts  have  been 
learned  from  experiment  and  the  effects  of  disease  and  iajury.  Their 
practical  importance  is  great. 

The  action  of  muscles  is  threefold.  (1)  By  their  tonic  contraction 
they  maintain  the  parts  in  a  certain  posture,  independently  of  volun- 
tary effort.  By  actual  contraction  they  (2)  produce  certain  move- 
ments, and  also  (3)  oppose  the  action  of  other  muscles  by  a  feebler 
contraction,  and  thus  steady  the  movement  that  results. 

The  complex  way  in  which  muscles  act  together,  and  modify  each 
other's  effect,  renders  the  subject  a  very  large  one.  Here  only  an 
outHne  can  be  given  of  the  more  salient  facts  concerning  the  most 
important  muscles.  The  reader  who  desires  to  pursue  the  subject 
further  can  do  so  ia  the  '  Physiologic  des  Mouvements '  of  Duchenne, 
whose  investigations  by  means  of  faradism  and  his  observations  on 
disease  were  so  careful  and  so  extensive  as  to  leave  little  but  confirmation 
to  subsequent  workers. 

In  the  following  account  the  nerve  that  gives  the  branch  to  the 
muscle  is  first  given,  and  then  the  number  of  the  nerve-root  or  roots — 
(C)ervical,  (D)orsal,  or  (L)umbar — from  which  the  nerve-fibres  come. 

Special  Thoeacic  Muscles. 

The  DiAPHEAGM  (C.  4  chiefly,  phrenic  nerves),  although  a  double  muscle  with 
Wo  nerves,  habitually  acts  as  a  whole,  the  two  halves  contracting  simultaneously 
and  diminishing  each  lateral  curve  of  the  arch.  The  central  tendon  descends 
but  little.  The  abdominal  viscera  are  depressed,  and  the  parietes  protmded. 
If  the  hand  is  placed  beneath  the  ribs,  the  descent  of  the  viscera  beneath  the 
diaphragm  can  be  felt.  When  the  diaphragm  contracts  alone,  as  when  the 
intercostals  are  paralysed,  or  the  phrenic  nerve  is  faradised,  the  ribs  to  which 
VOL.  I.  3 


34 


ACTION  AND  PAEALTSIS  OF  MUSCLES. 


the  muscle  is  attached  are  slightly  raised  during  its  action,  and  this  elevation 
causes  a  slight  expansion  of  the  thorax.  In  ordinary  breathing  this  expansion 
is  lost  in  the  action  of  the  intercostals.  In  paralysis  the  inspiratory  protmsion 
of  the  upper  part  of  the  abdomen  is  lost ;  it  even  recedes  during  inspiration 
instead  of  advancing,  and  a  descent  of  the  viscera  can  no  longer  be  felt  by  the  hand. 
There  often  results  an  alternation  in  the  respiratory  movements  of  the  thorax 
and  abdomen,  retraction  of  the  one  corresponding  to  protrusion  of  the  other. 

Steeno-MASToid  (spinal  accessory  nei've  C.  2 — 5  or  6,  also  branches  from  upper 
tervlcal  plexus),  passing  from  the  sternum  and  adjacent  part  of  the  clavicle  to 
the  mastoid  process,  inclines  the  head  towards,  and  rotates  the  face  from,  the 
side  on  which  the  muscle  contracts.  Both  muscles  together  support  the  head  in 
the  vertical  position,  and  if  it  is  bent  back,  they  bring  it  forwards  into,  but  not 
beyond  this  position.  Paralysis  of  one  muscle  has  no  influence  on  the  position 
of  the  head,  and  but  little  on  its  movements.  Other  muscles  supplement  the 
loss.  There  is  no  such  thing  as  a  "  paralytic  torticollis."  In  palsy  of  both 
muscles  the  head  can  be  balanced  in  the  vertical  position,  but  if  it  falls  back  it 
can  be  brought  forward  only  with  great  difficulty.  Each  sterno-mastoid  is 
associated  in  action  with  the  muscles  of  the  other  side ;  it  is  a  "  contra-lateral 
muscle."  For  instance,  in  using  the  right  arm,  the  head  is  tui'ned  to  the  right 
by  the  left  sterno-mastoid.     This  association  is  sometimes  reproduced  in  disease. 


Mtjscles  moving  the  TTppee  Limbs. 

Muscles  moving  the  Scapula  and  Shoxjldee-joint. — The  Trapezius 
spinal  accessory  (C.  2  to  6,  whether  also  the  lowest  C.  and  upper  D.  is  doubtful)* 
consists  of  three  parts.  The  first,  from  the  occipital  bone  to  the  outer  end  of 
the  clavicle,  is  rarely  used  except  in  breathing  (respiratory  portion — ^Duchenne). 


Fig.  5. — Paralysis  and  wasting  of  tra- 
pezius ;  alteration  in  contour  of 
shoulder  at  rest. 


Pig.  6. — Ditto  when  the  arms  are 
raised  (the  right  one  being  aided 
by  another  person). 


The  second  part  is  that  which  passes  from  the  lig.  nuchae,  lowest  cervical,  and 
upper  three  dorsal  spines,  downwards  and  outwards  to  the  acromion  and  outer 

*  Anatomists  now  believe  that  the  twigs  from  these  nerves  only  pass  through  the 
muscle,  and  tliat  it  is  wholly  supplied  by  the  spinal  accessory.  This  agrees  with  the 
fact  that  its  artion  is  chiefly  with  the  arm. 


MUSCLES   OF   UPPEE   LIMB. 


85 


part  of  tlio  Bpine  of  the  scapula.  The  lowest  part  passes  from  the  dorsal  spines 
below  the  third,  outwards  and  partly  upwards,  to  the  inner  half  and  base  of  the 
spine  of  the  scapula.  The  second  part  is  the  chief  elevator  of  the  scapula  and 
shoulder.  With  the  third  part  it  brings  the  scapula  towards  the  spine,  and  puts 
the  shoulder  back.  Both  parts  tend  to  rotate  the  scapula — acromion  up,  lower 
angle  out.  By  this  rotation  the  arm  is  carried  above  the  horizontal  level  to 
which  the  deltoid  raises  it.  Paralysis  of  the  highest  pai-t  has  little  influence  on 
the  movement  of  the  scapula,  but  causes  a  change  in  the  contour  of  the  neck 
(Fig.  5)  especially  conspicuous  on  deep  inspiration.  The  change  in  the  shape 
of  the  neck  is  very  great,  when  the  arms  are  raised,  if  the  whole  trapezius  is 
wasted  (Fig.  6).  In  palsy  of  the  middle  part  the  elevation  of  the  shoulder  is 
imperfect ;  in  that  of  the  third  part  the  scapula  is  farther  from  the  spine  than 
normal.  In  palsy  of  all  parts  the  scapula  becomes  rotated  (acromion  down, 
infeiior  angle  in)  by  the  weight  of  the  arm  and  the 
contraction  of  the  opponents  (Fig.  7).  The  rotation 
may  mask  the  displacement  outwards,  due  to  the 
paralysis  of  the  lowest  part.  If  the  clavicular  part 
remains,  there  may  bo  no  rotation,  but  the  scapula 
is  lower  than  normal. 

The  Rhomboids  (a  nerve  that  passes  through  the 
scalenus — C.  4  and  5)  first  rotate  the  scapula  on  the 
outer  angle,  moving  the  lower  angle  inwards,  and 
then  move  the  whole  scapula  upwards  and  inwards. 
In  strong  elevation  they  aid  the  trapezius,  which 
prevents  the  rotation  of  the  scapula.  The  rotatory 
action  aids  forcible  depression  of  the  raised  arm. 
The  muscles  also  fix  the  scapula  for  the  action  of  the 
teres  major.  Their  tone  helps  to  keep  the  scapula 
against  the  thorax  (opposing  the  pectoralis)  and  in 
its   vertical   position  (opposing    the   serratus),  and 

hence,  in  paralysis,  the  edge  of  the  scapula,  at  rest,   Yia,    7. Paralysis    and 

stands  out  a  little,  leaving  a  furrow,  and  the  scapula  is       wasting  of  deltoid  and 

slightly  rotated  (lower  angle  out).    Movement  is  but 

little  interfered  with  by  the  paralysis  of  the  rhomboids; 

but  the  movement  backwards  of  the  raised  arm  by 

the  teres  and  deltoid  is  feeble  for  want  of  the  fixation 

of  the  scapula. 

The  Levator  anguU  scapulae  (direct  branches  from  C.  3  and  4  or  5)  first  rotates 
the  scapula  on  the  outer  angle  and  then  raises  it.  The  muscle  is  usually 
paralysed  with  the  trapezius,  and  then  the  scapula  falls,  but  the  special  effect 
of  its  palsy  is  lost  in  that  of  the  trapezius.  If  the  levator  is  preserved,  and  the 
trapezius  paralysed,  there  is  great  rotation  of  the  scapula,  which  is,  as  it  were, 
suspended  by  its  inner  angle  (Fig.  7). 

Serratus  magnus  (posterior  thoracic  nerve — C.  5  and  6)  carries  the  scapula 
outwards,  forwards,  and  slightly  upwards  when  the  arm  is  put  forwards.  It 
tends  to  rotate  the  scapula  on  the  inner  angle  (acromion  up),  the  lower  fibres 
most  powerfully,  hut  this  rotation  is  prevented  by  the  rhomboids  and  levator 
anguli.  It  does  not  raise  the  shoulder  when  the  arm  is  hanging.  It  helps  to 
fix  the  scapula  when  the  posterior  fibres  of  the  deltoid  move  the  raised  arm 
back.  If  the  scapula  is  fixed  by  the  rhomboids,  the  serratus  can  act  on  the 
ribs,  and  aid  foi'ced  inspiration.  It  has  most  inspiratory  effect  when  the  arms 
are  elevated.     In  paralysis  there  may  be  little  change  in  the  position  of  the 


trapezius ;  rotation  of 
scapula,  from  weight  of 
arm,  in  consequence  of 
the  paralysis  of  the  tra- 
pezius; progressive  mui« 
cnlar  atrophy. 


36 


ACTION   AND  PARALYSIS   OF   MUSCLES. 


scapula  at  rest,  but  often  there  is  slight  rotation  (lower  angle  in)  from  the 
unopposed  tone  of  the  rhomboids.  When  the  arm  is  moved  forwards  by  the 
anterior  part  of  the  deltoid,  the  scapula,  no  longer  held  against  the  thorax  and 
moved  forwards  by  the  serratus,  is  rotated  on  its  vertical  axis  by  the  action  of 
the  anterior  part  of  the  deltoid  on  the  humerus,  and  of  the  middle  part  on  the 
scapula.  Thus  the  posterior  edge  recedes  from  the  thorax,  leaving  a  groove 
into  which  the  hand  can  sometimes  be  placed  (Fig.  8).  The  scapula  is  at  the 
same  time  rotated,  lower  angle  inwards  and  upwards.  Elevation  of  the  arm 
above  the  level  of  the  shoulder  is  much  weakened,  but  can  be  imperfectly 
effected  by  the  middle  part  of  the  trapezius.  Loss  of  the  serratus  weakens 
other  movements,  but  does  not  abolish  any.  Inspiratory  expansion  of  the  thorax, 
when  the  arms  are  raised,  is  distinctly  less  on  the  paralysed  side  (Poore). 


Fig.  8.— Paralysis  of  the  serratus  magnus ;  FiG.  9.— Paralysis  of  right  deltoid;  ele^ 
eversion  and  rot;ition  of  scapula  when  vatiou  of  shoulder  by  trapezius  on  an 
the  arm  is  put  forwards.  attempt   to    raise    the    arm,    which   is 

slightly  abducted  by  the  supraspiuatus. 

The  Deltoid  (circumflex  nerve,  from  the  brachial  plexus  posterior  cord — C.  4 
and  5)  abducts  the  humerus,  the  anterior  and  posterior  fibres  also  moving  the  arm 
forwai-ds  and  backwards  respectively.  The  arm  is  raised  least  by  the  posterior, 
and  most  by  the  anterior  fibres,  but  even  the  latter  only  elevate  it  to  a  right 
angle  with  the  trunk.  Hence,  if  raised  by  the  anterior  fibres,  and  then  moved 
back  by  the  posterior,  it  is  at  the  same  time  depressed.  Elevation  above  a 
right  angle  is  by  rotation  of  the  scapula  (trapezius  and  serratus).  These 
muscles  also  fix  the  scapula  for  the  deltoid,  preventing  the  rotation  (acromion 
down,  lower  angle  in)  that  the  deltoid  acting  alone  would  cause.  In  paralysis, 
abduction  of  the  arm,  direct,  forwards,  and  backwards,  is  almost  lost.  All  the 
abduction  that  remains  is  a  trifling  movement  by  the  supraspinatus.  An 
attempt  to  abduct  results  in  rotation  of  the  scapula  and  elevation  of  the 
shoulder  (Fig.  9)  from  an  excessive  innervation  of  tlie  associated  trapezius  and 
serratus,  which,  as  we  have  seen,  fix  the  scapula  when  the  deltoid  acts.  Para- 
lysis of  single  parts  of  the  deltoid  causes  loss  of  the  corresponding  movements 
of  the  arm,  but  if  the  middle  part  only  is  paralysed,  there  is  still  a  limited 


MUSCLES    OF  UPPER   LIMB.  37 

power  of  direct  abduction  by  tbe  conjoined  contraction  of  tbe  anterior  and 
posterior  parts,  aided  by  the  supraspinatus. 

The  Supraspinatus  (suprascapular  nerve — C.  4  and  5)  ahducta  the  arm, 
moves  it  forwards,  and  rotates  it  in.  It  thus  aids  the  deltoid.  Isolated  para- 
lysis of  the  supraspinatus  has  little  influence  on  movement  or  position ;  but  if 
the  deltoid  is  also  paralysed,  the  head  of  the  humerus  falls  away  from  the 
acromion  far  more  than  when  the  deltoid  is  paralysed  alone. 

The  Jn/raspinaitts  (suprascapular  nerve— C.  4  and  5)  rotates  the  humerus 
outwards,  and  in  paralysis  this  movement  is  lost.  A  diflaculty  in  writing  is 
produced,  the  movement  along  the  line  being  by  this  rotation  of  the  humeras. 

The  Teres  minor  (cii'cumflex  nerve — C.  5)  has  a  similar  action  to  the  infra- 
spinatus, and  its  palsy  has  a  similar  effect. 

The  Subscapularis  {short  subscapular  nerve,  from  the  fifth  and  sixth  cervical) 
rotates  the  humerus  in,  and  its  paralysis  lessens  this  movement. 

The  Latissimus  dorsi  (long  subscapular  nerve,  from  the  brachial  plexns, 
posterior  cord — C.  7)  lowers  the  raised  arm,  and  puts  it  back;  the  upper  pai-t 
adducts  the  scapula,  the  lower  depresses  the  shoulder  by  acting  on  the  humeras, 
which  it  tends  to  drag  out  of  the  socket.  It  inclines  the  trunk  a  little,  and 
both  muscles  together  extend  the  trunk.  In  paralysis,  forcible  backward 
depression  of  the  raised  arm  is  lost,  and  the  shoulder  cannot  be  put  back 
without  being  also  raised  (by  the  trapezius). 

The  Pectoralis  major  (anterior  thoracic  nerves  from  the  brachial  plexus, 
outer  and  inner  cords — C.  5,  6,  and  7)  consists  of  two  muscles,  the  action  of  the 
clavicular  and  sternal  parts  being  different.  The  clavicular  (which  arises  also 
from  the  highest  part  of  the  steinum),  if  the  arm  is  hanging,  brings  the 
shoulder  forwards  and  upwards,  as  if  shivering;  if  the  arm  is  raised,  it  is 
brought  forwai-ds  and  lowered  to  the  horizontal  position.  The  muscle  is  thus 
concerned,  Duchenne  says,  in  the  "cut  "  of  the  swordsman  and  the  benediction 
of  the  priest.  The  sternal  portion  lowers  the  raised  arm  from  every  position, 
and  if  the  arm  is  hanging,  it  draws  the  shoulder  down.  Paralysis  of  the  upper 
part  has  little  effect  on  the  movement  of  the  arm,  because  the  anterior  fibres  of 
the  deltoid  have  the  same  action.  It  is  easily  recognised  by  making  the  patient 
put  his  arms  in  front  of  him  and  press  the  palms  together.  In  paralysis  of  the 
lower  part,  even  with  the  latissimus,  the  raised  arm  can  still  be  lowered  accu- 
rately by  the  weight  of  the  arm  and  relaxation  of  the  elevators,  but  it  cannot 
be  lowered  against  even  a  slight  resistance.  Thus  a  blacksmith  with  this  defect 
could  wield  a  heavy  hammer,  but  could  not  blow  the  bellows  by  pulling  down- 
wards a  cord  (Duchenne). 

The  Teres  major  (short  subscapular  nerve  from  the  brachial  plexus 
posterior  cord — C.  7)  appi'oximates  the  humeras  and  the  outer  edge  of  the 
scapula,  by  bringing  the  former  to  the  side  of  the  trunk,  and  rotating  the  lattei-. 
The  simultaneous  contraction  of  the  levator  anguli  and  rhomboids,  fixing  the 
mner  angle,  causes  this  rotation  to  raise  the  prominence  of  the  shoulder. 
Hence,  in  a  forcible  elevation  of  the  shoulder,  the  arm  is  pressed  against  the 
side,  the  lower  fibres  of  the  latissimus  and  pectoralis  major  aiding  the  adduction. 
The  teres  cannot  alone  put  the  arm  behind  the  trunk.  In  paralysis,  the  eleva- 
tion of  the  shoulder,  with  the  arm  against  the  side,  is  lost. 

Muscles  moving  the  Foeeaem. — Triceps  (musculo-spiral  nerve — C.  6  and 
7). — The  long  head  has  an  action  similar  to  the  teres,  but  feebler.  It  contracts 
when  the  arm  is  forcibly  lowered,  and  prevents  the  displacement  downwards  of 
the  head  of  the  humerus  by  the  actual  depressors,  the  latissimus  and  pectoralis. 
All  parts  extend  the  elbow,  the  long  head  with  less  force  than  the  others,  but  its 


38 


ACTION  AND  PAKALTSIS  0¥  MUSCLES. 


action  on  the  shoulder-joint,  just  mentioned,  ia  important,  because  forcible 
depression  of  the  raised  arm  is  often  associated  with  extension  of  the  elbow.  In 
paralysis  of  the  triceps  the  elbow  can  only  be  extended  by  the  weight  of  the  fore- 
arm, and  extension  against  gravitation  is  impossible.  Thus  a  man  with  paralysis 
of  the  triceps  cannot  raise  his  hat  in  the  customary  manner.  Flexion  of  the 
elbow  is  uncertain,  on  account  of  the  loss  of  the  antagonistic  steadying  force. 
The  Brachialis  anticus  (musculo-cutaneous  and  musculo-spiral  nerves)  flexes 


Triceps  (long  head)  — 

Triceps  (inner  head) 
Ulnar  n.  < 


Flex,  carpi  ulnar. 
Flex.  dig.  profund. 

Flex.dig.  sub.  (ir&  III) 

Flex,  digit,  sub.  (I  &IV) 
Ulnar  n. 

Palm  brev, 

Abd.  niiniiu.  digit. 

Flex.  min.  aitr. 

Oppon.  min.  dig. 

Lumbricales 


Deltoid 
(ant.  half) 


Mus.-cut.  n. 
Biceps 
Brach.  ant. 

>  Median  n. 
Supinator  long. 
Pronator  teres 
Flexor  carpi  radialis 

Flex,  digitor  sublim. 

Flex.  long,  pollicis 
Median  n. 

Adductor  pollicis 
Opponens  pollicis 

Flex.  brev.  pollicis 

Adductor  pollicis 


Fig.  10. — Motor  points  for  the  arm,  inner  side  (from  Erb).     The  points  at  which  the 
nmsfles  :iiid  nervps  can  most  effectively  be  stimulated. 


MUSCLES  OP    UPPER  LIMB, 


39 


the  elbow  simply.  Its  rare  isolated  palsy  has  little  effect,  since  it  is  supple- 
mented by  the  biceps  and  supinator  longus. 

The  Biceps  (musculo-cutaneous  nerve — C.  4,  5,  and  6)  supinates  the  forearm 
if  it  is  pronated,  and  then  flexes  the  elbow.  In  paralysis  the  flexion  can  still  be 
effected,  but  the  traction  on  the  humerus  causes  pain  at  the  shoulder,  from  the 
loss  of  the  support  of  the  long  head  of  the  biceps. 

The  Supinator  longus  (musculo-spiral  nerve — C.  4  and  5)  places  the  forearm 
midway  between  pronation  and  supination,  and  then  flexes  the  elbow.  If  it  is 
paralysed  there  is  a  tendency  for  supination  to  accompany  flexion  (biceps) ;  and 
if  the  brachialis  anticus  is  also  paralysed,  the  elbow  can  only  be  flexed  when 
the  foreann  is  supinated. 

If  the  three  direct  flexors  of  the  elbow  are  paralysed,  feeble  flexion  is  still 
possible  by  the  extensors  of  the  wrist,  which  cross  the  elbow-joint,  but  only  after 
the  forearm  has  been  pronated  and  the  wrist  over-extended. 


Deltoid 
(posterior  half) 


Muse. -spiral  n. 
Brachialis  ant. 

Supiuator  longus 
Extensor  radial,  long. 
Extensor  radial,  brev. 


Extensor  digit.  -I 
Extensor  indicis 

Ext.  OS.  met.  pel. 
Extensor  pr.  intern,  pol. 


Dorsal  interossei  J 
(I  and  II)        1 


Triceps  (long  Lead) 


Triceps  (outer  head) 


Extensor  carpi  uln. 
Supinat.  brev. 

Extens.  minim,  digiti 
Extensor  indicis 

Extens.  long.  poll. 


Abduct,  minim,  digiti 

}  Dorsal  interossi^i 
(III  and  IV) 


Fig.  11. — Motor  points  for  the  wrm,  outer  side  (Erb), 


40  ACTION  AND  PARALYSIS  OF  MUSCLES. 

The  Supinator  hrevis  (musculo-spiral  nerve  by  posterior  interosseous  branch— 
C.  5)  is  the  only  simple  supinator.  If  it  is  paralysed,  supination  can  still  be 
efEected  by  the  biceps,  and  also  by  the  supinator  longus  as  far  as  midw^ay  between 
pronation  and  supination. 

Pronators. — The  p.  teres  and  p.  quadratus  (median  nerve — C.  6  and  7)  both 
pronate  strongly,  and  their  palsy  causes  loss  of  this  movement,  but  pronation  to 
the  mid-position  is  still  possible  by  the  supinator  longus. 

Muscles  moving  the  Hand. — Flexors  of  Wrist. — The  F.  carpi  ulnaris 
(ulnar  nerve)  and  F.  carpi  radialis  (median — C.  7  and  8)  flex  the  wrist.  The 
ulnar  flexor  tends  to  turn  the  supinated  hand  still  more  out,  but  neitlier  moves 
the  wrist-joint  laterally.  Flexion  indeed  hinders  the  lateral  movements  of  the 
wrist,  in  consequence  of  the  shape  of  the  articular  surfaces.  The  f.  c.  ulnaris 
flexes  the  fifth  metacai-pal  bone  on  the  carpus  as  well  as  the  wrist-joint.  In 
paralysis,  flexion  of  the  wrist  can  only  be  effected  by  the  flexors  of  the  fingers 
when  these  are  extended.  Extension  of  the  wrist  is  unsteady  from  the  loss  of 
the  antergic  contraction  of  the  flexors  (see  p.  10). 

Extensors  of  Wrist. — Extensor  carpi  radialis  hrevis  et  longus  ;  E.  c.  ulnaris 
(musculo-spiral  nerve  and  its  posterior  interosseous  branch — C.  6  and  7).  The 
short  radial  is  a  direct  extensor ;  the  long  radial  and 
the  ulnar  move  the  hand  laterally  as  well.  In  para- 
lysis of  all  thi-ee  extensors  the  wrist  can  only  bo 
extended  by  the  extensors  of  the  fingers  when  the 
phalanges  are  flexed.  In  loss  of  the  short  radial, 
direct  extension  is  still  possible  by  the  long  radial  and 
ulnai',  and  lateral  extension  by  one  of  these  alone. 
If  either  is  paralysed,  together  with  the  short  radial, 
direct  extension  is  lost,  and  only  lateral  extension,  in 
the  direction  of  the  remaining  muscle,  is  possible. 
Pai'alysis  of  either  lateral  extensor,  long  radial  or 
ulnar,  leads  to  permanent  deviation  of  the  wrist  in 
the   direction   of  the  remaining   muscle    (Fig.    12). 

Pig.   12. Paralysis  of      The  loss  of    the  long  radial  is  more    serious    than 

the  long  radial  ex-  that  of  the  ulnar,  because  the  radial  lateral  move- 
tensor  of  the  wnst  in  a  ixient  is  of  more  importance,  being  needed  for  the 
dSon'S'thf  hS  convenient  motion  of  the  hand  to  the  mouth.  Para- 
towards  the  ulnar  side.  lysis  of  the  extensor  impairs  flexion  of  the  fingera, 
(After  Duchenue.)  from  the  great  shortening  of  the  course  of  the  ten- 

dons by  the  flexion  of  the  wrist  that  occurs.  The 
ulnar  extensoi*  acts  also  synergically  with  the  extensor  of  the  metacarpal 
bone  of  the  thumb,  as  may  be  noted  if  the  finger  is  placed  on  the  tendon  beneath 
the  styloid  process  of  the  ulna.  Hence,  in  paralysis  of  this  extensor,  the  hand 
deviates  laterally  when  the  thumb  is  strongly  extended. 

Extensors  of  the  Fingers. — Extensor  communis  digitorum ;  E.  indicis  ; 
E.  minimi  digiti  (musculo-spiral  nerve — C.  6  and  7).  The  common  extensor 
moves  the  fingers  and  then  the  wrist.  When  the  muscle  is  faradised,  the 
extension  begins  at  the  distal  phalanges,  and  these  become  flexed  again,  when 
the  hand  is  extended  beyond  the  plane  of  the  forearm,  by  the  tonic  force  of  the 
flexors,  the  course  of  their  tendons  being  elongated  by  the  extension  of  the  wrist. 
Moi'eover  the  muscle  has  little  action  on  the  last  two  phalanges,  since  they 
cannot  be  extended  by  the  long  extensor,  if  the  interossei,  their  proper  extensors, 
are  paralysed.  During  extension  by  the  communis  the  fingers  are  separated 
from  the  second.     The  extensors  of  the  first  and  last  fingers  have  a  similar 


MUSCLES  OP  UPPER  LIMB. 


41 


extensor  action,  but,  in  addition,  they  adduct  their  respective  fingers  towards  the 
middle  finger.  In  paralysis,  the  extension  of  the  fingers  is  impossible;  but  if 
the  proximal  phalanges  are  passively  extended, the  middle  and  distal  joints  can 
be  extended  by  the  interossei.  For  the  lateral  movements  of  the  digits,  exten- 
Bion  of  the  proximal  phalanges  is  essential,  and  hence  these  movements  are 
lost,  but  they  can  be  performed  if  the  proximal  phalanges  are  passively  extended. 
The  posture  of  the  fingers  due  to  contraction  of  the  palmar  fascia  resembles 
that  in  palsy  of  the  long  extensor  (see  Fig.  13),  but  an  examination  of  the 
palm  shows  the  cause  of  the  flexion. 

Flexors  of  Fingers. — F.  sublimis  (median  nerve — all  fibres  C  7  and  8) ;  F. 
profundus  (median  and  ulnar  nerves).  These  muscles  flex  chiefly  the  second 
and  third  phalanges,  the  first  phalanx  being  flexed  by  the  interossei.  The 
superficial  muscle  flexes  the  second  phalanx  on  the  first,  the  deep  flexes  both. 
The  action  on  the  first  phalanx  is  confined  to  extreme  flexion  of  the  fingers,  and  is 
the  less,  the  more  the  wrist  is  flexed.  But  if  the  flexion  of  the  middle  and  distal 
phalanges  is  prevented  the  first  is  strongly  flexed.  In  extreme  shortening  of 
the  course  of  the  tendons  by  flexion  of  the  wrist,  the  action  on  the  fingers  is 
very  feeble,  evidence  of  a  normal  antergic  action  of  the  extensors  of  the  wrist. 


Fig. 13 


Fig.  14. 


Fig.  13. — Posture  of  the  hand  in  contraction  of  the  palmar  fascia,  resembling 
that  in  paralysis  of  the  long  extensors  of  the  finger. 

Fig.  14.  —  Paralysis  of  the  fibres  of  the  flexor  sublimis  which  act  on  the  two 
middle  fingers :  twelve  years'  duration.  The  second  phalanges  of  these  fingers 
are  bent  backwards  and  subluxated  from  the  contracture  of  the  unopposed 
interossei,  while  the  last  plialanges  are  kept  in  position  by  the  unaffected 
flexor  profundus.     (After  Duchenne.) 


When  the  extensor  of  the  fingers  is  in  strong  action,  extending  the  proximal 
phalanges,  the  action  of  the  flexors  on  the  second  and  third  joints  is  very  strong 
(tearing  position).  In  paralysis  of  these  muscles  the  power  of  flexing  the  last 
two  joints  is  lost,  but  the  interossei  still  flex  the  metacarpo-phalangeal  joints. 
Paralysis  of  the  deep  flexor  alone  causes  merely  loss  of  the  power  of  flexing 
the  distal  joint,  but  this  impairs  many  movements,  such  as  playing  on  the 
piano.  In  paralysis  of  these  muscles,  the  unopposed  tone  of  their  opponents, 
the  interossei,  which  extend  these  joints,  leads  in  time  to  over  extension,  and, 
with  repeated  passive  pressure  in  using  the  fingers,  may  even  produce  a  sub- 
luxation backwards.  In  palsy  of  the  sublimis  this  effect  is  chiefly  seen  at  the 
middle  joint  (Fig.  14),  in  that  of  the  profundus  at  the  distal  joint. 

Interossei  and  Lumhricales  (ulnar  nerve,  except  the  outer  two  lurabricales, 
which  are  supplied  by  the  median — C.  8  and  D.  1). — The  interossei  abduct  and 


42 


ACTION  AND  PARALYSIS  0^  MUSCLES. 


adduct  the  fingers,  but  only  when  these  are  extended  at  the  metacarpo« 
phalangeal  joints,  and  some  effort  is  required  for  adduction,  since  the  tendency 
of  the  long  extensor  is  to  separate  the  fingers,  and  this  influence  has  to  be 
overcome.  They  also  extend  the  second  and  third  phalanges  on  the  first,  and 
flex  the  first  on  the  metacarpal  bones.  The  lumbricales  aid  the  flexor-extensor 
action  of  the  interossei,  but  do  not  move  the  fingers  laterally.  The  opposite 
action  of  the  forearm  muscles  and  of  the  interosseous  extensors  and  flexors  is  veiy 
important.  Their  synergic  action  steadies  movements,  and  in  many  actions  they 
contract  alternately.  Thus  in  making  a  down-stroke  with  a  pen  or  pencil  the 
long  flexors  bend  the  last  two  joints ;  while  in  making  an  up-stroke  these  are 
extended,  and  the  metacarpo-phalangeal  joint  is  flexed,  by  the  interossei.  In 
paralysis  of  these  muscles  the  lateral  movements  are  lost,  but  a  slight  abduction 
and  adduction  of  the  index  can  still  be  effected  by  its  long  extensors.  Only  the 
first  phalanx  can  be  extended,  and  flexion  is  almost  confined  to  the  last  two 
phalanges.  The  first  two  lumbricales,  being  supplied  by  the  median  nerve, 
often  escape  when  the  other  muscles  are  paralysed  by  an  injury  to  the  ulnar 
nerve,  and  they  aid  the  others  when  these  are  merely  weak;  hence  the  index 
and  middle  fingers  seem  to  recover  before  the  others  (Fig.  15).    The  position  of 


Fia.  15. 


Fia.  16. 


Fig.  15. — Recent  incomplete  paralvsis  of  the  interossei  from  a  punctured 
wound  of  the  uluiir  nerve  at  the  wrist:  iittempt  to  extend  fingers.  The  loss 
of  extension  of  the  last  two  phalanges  is  cliieHy  marked  in  the  third  and  fourth 
fingers,  from  the  influence  of  the  lumbricales  (supplied  by  the  median)  on  the 
others.     (After  Duclienne.) 

Pig.  16. — P.iral\sis  of  the  interossei  (ulnar  uerve)  slight  in  degree:  attitude 
of  fingers  at  rest. 


the  hand  at  rest  becomes  altered.  Normally  there  is  slight  flexion  at  all  joints 
by  the  tone  of  the  muscles,  interossei  and  long  flexors.  In  paralysis  the  first 
phalanx  is  in  a  line  with  the  metacarpal  bones,  while  the  other  phalanges  are 
flexed,  the  middle  more  than  the  distal  (Fig.  16).  In  action  this  flexion  is 
always  increased,  the  metacarpo-phalangeal  joints  become  over-extended,  and 
the  other  joints  strongly  flexed  (Fig.  17).  Gradually  the  hand  assumes  this 
posture  even  at  rest  (Fig.  18),  and  ultimately  the  posture  becomes  warped  into 
a  deformity  by  the  over-extension  of  the  first  phalanges,  and  extreme  flexion  oE 
the  others,  due  to  the  contracture  of  the  long  extensor  and  of  the  flexors;  the 
tendons  of  tliese  muscles  stand  out  conspicuously  on  the  back  and  in  the  palm, 
and  a  claw-like  attitude  is  developed,  the  "  main  en  gi'iffe  "  (Figs.  19  and  20). 
Changes  in  the  articulations  may  ultimately  limit  even  passive  movement. 

Muscles  of  the  Thumb  — Extensor  secundi  internodii  pollicis  (musculo- 
Bpiral  nerve,  posterior  interosseous  branch— C.  8  and  D.  1)  extends  both 
phalanges,  and  moves  the  whole  thumb  backwards  and  from  the  fingers,  so  as 
to  bring  it  behind  the  plane  of  the  metacarpus.  It  may  ultimately  extend  the 
wrist-joint,  but  it  never  supinates.     It  is  not  used  in  extending  the  thumb  when 


MUSCLES  OP  UPPER  LIMB. 


43 


this  is  opposed  to  the  first  finger.  In  paralysis,  the  metacarpal  hone  of  the 
thumb  is  slightly  flexed  on  the  carpus,  and  is  inclined  forwards.  The  second 
phalanx  is  flexed  on  the  first,  and  can  only  be  extended  (by  the  abductor  and 
outer  part  of  the  short  flexor)  when  the  metacarpal  bone  is  adducted  and  the 
fii-st  phalanx  is  flexed.  The  constant  flexion  of  the  second  phalanx  interferes 
with  the  movement  of  the  index  finger,  unless  the  patient  remembers  to  move 
the  thumb  out  of  the  way  by  the  extensor  of  the  metacarpal  bone.  Writing  is 
not  interfered  with  because  the  muscle  i8  not  concerned  in  extension  with 
opposition. 


Fiff.  17 


Fia.  18. 


Fia.  19. 


Fig.  20. 


PiO.  17. — Attempt  to  unbutton  waistcoat  by  the  hand  shown  in  the  last 
figure ;  extreme  flexion  of  the  last  two  phalanges,  and  extension  of  the  first,  on 
the  attempt  to  use  the  fingers.     (From  nature.) 

Fig.  18.— Old-standing  palsy  of  interossei  and  thenar  muscles,  showing  the 
over-extension  of  the  first  and  flexion  of  the  last  two  phalanges. 

Fig.  19. — Paralysis  of  all  the  intrinsic  muscles  of  the  hand  and  of  the  long 
flexor  of  the  thumb,  in  conseqtience  of  an  injury  to  the  brachial  plexus  in  dis- 
location of  tlie  shoulder.  Tlie  fingers  present  the  claw-like  attitude  j  the  thumb 
is  in  extension.     (After  Duchenne.) 

Fig,  20. — Piiralysis  of  the  ulnar  nerve  from  a  wound  at  the  wrist  (indicated 
in  the  figure).  Extreme  daw-like  hand  from  the  unopposed  contraction  of  the 
coimnon  extensor  and  long  flexors  of  the  fingers  and  thumb.     (After  Duchenne.) 


The  Extensor  primi  internodii  pollicis  (musculo-spiral  nerve,  post-inteross. 
branch — C.  8  and  D.  1)  is  the  true  abductor  of  the  thumb.  It  moves  the 
metacarpal  bone  outwards,  and  extends  the  first  phalanx.  It  would  move  the 
whole  hand  in  the  same  direction  as  the  thumb,  were  not  this  tendency  counter- 
acted by  the  anteigic  contraction  of  the  extensor  carpi  ulnaris  (q.  v.).  It  does 
notpronate  or  supinate.  In  paralysis  abduction  of  the  metacarpal  bone  is  less 
than  normal.  There  is  an  undue  flexion  of  the  firat  phalanx,  and  the  meta- 
carpal bone  is  flexed  on  the  carpus,  so  that  the,  thumb  is  drawn  towards  the 
palm.  The  loss  of  this  muscle  is,  however,  compensated  to  a  considerable 
extent  by  other  muscles. 


44  ACTION  AND  PARALYSIS  OF  MUSCLES. 

The  Extensor  ossis  metacarpi  pollicis  (musculo-spiral  nerve,  post-inteross. 
branch— C.  8  and  D.  1)  is  really  the  long  abductor  o£  the  thumb.  It  moves 
the  metacarpal  bone  outwards  and  forwards,  flexing  it  on  the  carpus,  and  then 
flexes  the  wrist  with  slight  pronation.  It  thus  moves  the  thumb  as  much 
forwards  as  outwards.  In  paralysis  the  metacarpal  bone  is,  at  rest,  less 
inclined  forwards  than  noi'mal,  and  somewhat  abducted,  but  the  first  phalanx  is 
in  its  normal  position.     Movement  of  the  thumb  is  but  little  interfered  with. 

In  combined  palsy  of  the  extensors  of  the  first  phalanx  and  of  the  metacarpal 
bone  the  thumb  becomes  adducted,  and  is  parallel  to  the  radius.  The  first 
phalanx  is  slightly  flexed  by  the  tlienar  muscles. 

The  Thenar  muscles  constitute  two  groups:  (1)  The  short  abductor  and 
outer  portion  of  the  short  flexor  (median  nerve — 0.  8  and  D.  1)  move  the 
metacarpal  bone  forwards  and  inwards  (flexing  the  first  phalanx),  incline  it 
outwards,  and  rotate  it  inwards,  so  as  to  place  its  palmar  aspect  opposite  the 
fingers.  The  second  phalanx  is  ultimately  extended.  If  the  metacarpal  bone 
is  previously  abducted  the  movement  is  greatei",  and  amounts  to  circumduction. 
(2)  The  adductor  and  inner  part  of  short  flexor  (ulnar  nerve — 0.  8  and  D.  1) 
go  to  the  inner  side  of  the  first  phalanx.  The  metacarpal  bone  is  moved 
towards  that  of  the  second  finger:  if  previously  flexed,  it  is  extended;  if  pre- 
viously opposed  to  the  index,  it  is  moved  a  little  outwards.  The  phalanges 
follow  the  movements  of  the  metacarpal  bone,  but  the  flrst  is  slightly  flexed 
and  the  second  is  extended,  as  the  fingers  are  by  the  interossei. 

The  Opponens  pollicis  (median  nerve — C.  8  and  D.  1)  flexes  the  metacarpal 
bone  on  the  carpus,  and  abducts  it,  but  this  movement  is  insuflScient  to  oppose 
the  thumb  to  the  index ;  the  conjoint  action  of  the  abductor  is  necessary  (see 
above). 

Fia.  21.  Fia.22.  Fia.  23. 


Fig.  21. — Normal  position  of  the  thumb  (for  compprison  with  the  succeeding 
''  figures). 

Fig.  22. — Position  of  the  hand  in  long-standing  paralysis  and  wasting  of  the 
thenar  muscles.  Under  the  influence  of  the  long  extensDr  the  metacarpal  bone 
01  the  thumb  has  been  brought  into  the  same  position  as  the  other  metacarpal 
bones,  being  rotated  slightly,  so  that  tlie  bac-k  of  the  thumb  is  in  the  plane  of 
the  back  of  the  hand,  like  the  hand  of  .tlie  ape.     (After  Duchenne.) 

Fig.  23,  from  another  case,  shows  the  same  condition,  but  still  greater  dis- 
placement of  the  metacarpal  bone  has  taken  place,  from  the  greater  contraction 
of  the  extensor.     (After  Duchenne.) 

The  Flexor  longus  pollicis  (median  nerve — C.  7  and  8,  and  D.  1)  flexes  the 
second  phalanx  forcibly  and  the  first  feebly.  It  has  no  action  on  the  metacarpal 
bone.  It  is  used  in  writing  (malting  a  stroke  towards  the  body)  and  in  picking 
up  a  small  object,  &c.  In  paralysis  this  flexion  is  lost,  and  with  it  these  actions ; 
if  an  object  is  held  between  the  tips  of  the  thumb  and  forefinger,  the  last 


MUSCLES   OP  LOWER  LIMB. 


phalanx  of  the  thumh  is  bent  back.    Other  movements  of  the  thumb  are  not 
interfered  with. 

If  all  the  thenar  muscles  are  paralysed  the  metacarpal  bone  is  in  the  plane  of 
the  index,  and  drawn  towards  it  by  the  extensor  of  the  second  phalanx,  which 
moves  the  metacarpal  bone  inwards  and  backwards.  The  whole  thumb  corre- 
sponds with  the  metacarpal  bone  in  position,  the  phalanges  being  normal.  In 
paralysis  oE  the  short  abductor  and  flexor  the  second  phalanx  canaot  be  extended 
unless  the  metacarpal  bone  is  abducted.  These 
short  muscles  normally  prevent  the  abduction 
that  the  extensor  of  the  second  phalanx  tends 
to  produce.  The  latter,  moreover,  prevents 
undue  adduction  when  the  special  abductors 
are  paralysed,  but  brings  the  metacarpal  bone 
into  the  plane  of  the  index.  Thus  the  posture 
of  the  hand  at  rest  resembles  that  of  the  hand 
of  an  ape  (Figs.  21  and  22).  If  the  abductor 
and  opponens  are  paralysed,  the  tips  of  the 
thumb  and  fingers  can  only  be  brought 
together  by  flexing  the  last  phalanges  of  the 
digits  (Fig.  24).  Then  the  thumb  can  be 
brought  into  contact  with  the  finger  by 
means  of  the  short  flexor,  which  inclines  the 
metacai-pal  bone  sufficiently  to  effect  this, 
although  not  enough  for  the  tip  of  the  first 
finger  to  touch  the  thumb  when  its  phalanges 
are  extended. 

If  the  short  flexor  is  paralysed,  the  thumb 
can  still  be  opposed  ,to  the  first  two  fingei-s 
by  the  abductor,  but  it  cannot  be  opposed  to 
the  last  two  fingers  on  account  of  the  defi- 
cient lateral  inclination  of  the  thumb,  which 
should  be  produced  by  this  muscle.  Writing 
is  easy  by  means  of  the  short  abditctor, 
whereas  if  this  is  lost,  although  the  tliurab 
can  be  opposed  to  each  of  the  fingers,  writing 
is  much  interfered  with. 

If  all  the  thenar  muscles  are  paralysed,  a  certain  amount  of  opposition  of  the 
thumb  and  fingers  is  still  possible,  by  means  of  the  flexion  of  the  last  phalanges 
of  the  thumb  and  fingers.  If  all  are  paralysed  except  the  adductor,  objects  can 
still  be  held  between  the  thumb  and  side  of  the  palm. 


Fis.  24. — Paralysis  of  the  ab- 
ductor brevis  and  opponens 
poUicis.  From  the  waut  of 
these  muscles  the  thumb  can 
only  .  be  brought  in  contact 
with  the  tip  of  the  index  by 
strong  flexion  of  the  last  two 
phalanges  of  the  fingers,  other- 
wise the  tip  of  the  thumb 
only  reaches  the  middle  of 
the  second  phalanx.  (After 
Duchenne.) 


Muscles  .  op  the  Lowee  Limb. 

Muscles  moving  the  Hip-joint. — The  Gluteus  maximus  (inferior  gluteal 
nerve,  small  sciatic,  and  a  special  branch  from  sacral  plexus — L.  4  and  5,  S.  1) 
extends  the  hip-joint,  and  freely  rotates  the  thigh  outwards.  It  is  the  most 
powerful  extensor  of  the  hip,  and  it  is  chiefly  used  when  a  forcible  extension 
is  required,  and  the  joint  has  been  previously  flexed.  It  is  employed,  not 
in  standing,  or  in  walking  on  level  ground,  but  in  going  upstairs,  or  uphill,  and 
in  rising  from  a  seat.     When  it  is  paralysed  these  movements  are  difficult. 

The  Gluteus  medius  (gluteal  nerve— L.  4  and  5,  S.  1)  is  the  chief  abductor. 
All  parts  of  the  muscle  have  this  action,  but  in  addition  the  anterior  third 


46 


ACTION  AND  PAEALTSIS  OP  MUSCLES. 


moves  the  thigh  forwards  and  rotates  it  inwards,  while  the  posterior  third 
moves  it  backwards  and  rotates  outwards.  The  successive  action  of  the  several 
parts  causes  circumduction.  The  Gluteus  minimus  (gluteal  nerve)  has  pro- 
bably the  same  action.    In  paralysis,  abduction  and  circumduction  are  lost;  in 


Crural  n. 


Adductor  mag. 
Adductor  long: 


Tens  T  vagiiiEe  femovi«, 

Sartorius. 

Qundriceps. 
Rectus  femoris. 

Vastus  extern. 


Vastus  inter 


Pia.  25. — Motor  points  on  the  front  of  the  thigh  (Erb), 


standing  on  the  other  foot  the  pelvis  is  inclined  on  the  affected  side,  and  hence, 
in  walking,  there  is  an  oscillation  of  the  trunk,  which  becomes  very  con- 
siderable if  the  muscles  of  both  sides  are  affected.  Moreover  the  unopposed 
tone  of  the  outward  rotators  produces  a  permanent  rotation  of  the  leg,  so  that 
the  toes  are  directed  outwards,  and,  from  the  altered  position  of  the  foot,  the 
propulsion  of  the  body  in  walking  is  deficient. 

The  Pyriformis,  Gemelli,  Obturator  internus,  and  Quadratus  femoris 
(special  nerves  from  the  sacral  plexus,  but  from  L.  5)  all  rotate  the  thigh  out- 
wards, and  the  first-named  muscle,  in  addition,  carries  the  thigh  obliquely 
backwards  and  outwards,  in  the  same  way  as  the  posterior  fibres  of  the  gluteus 
medius.  In  paralysis  of  these  muscles  external  rotation  is  impossible,  and  the 
unopposed  tone  of  the  internal  rotators  (anterior  fibres  of  the  gluteus  medius 
and  minimus)  causes  the  leg  and  foot  to  be  habitually  turned  inwards. 

The  Psoas  (special  lumbar  nerve,  L.  2  and  3)  and  Iliacus  (anterior  crural 
nerve  L.  2  and  3)  flex  the  hip-joint,  and,  in  doing  so,  cause  also  a  slight 
rotation  outwai'ds.  In  paralysis,  flexion  is  lost,  and  the  use  of  the  leg  in 
walking  becomes  impossible. 

The  Tensor  vagina  femoris  has  a  slight  power  of  flexing  the  hip,  and  at  the 
same  time  rotates  the  thigh  in.     It  normally  counteracts  the  tendency  of  the 


MUSCLES   OF  LOWEE  LIMB.  47 

ilio-psoas  to  rotate  outwards.  If  it  is  paralysed,  there  is  a  tendency  for  the  foot 
to  turn  out  when  it  is  being  brought  forward  in  the  act  of  walking. 

Adductors  of  the  Thigh  (all  are  innervated  from  the  same  roots,  L.  2  and  3). 
—The  Pectineus  (obturator  nerve)  causes  an  oblique  movement  forwards  and 
inwards,  i.  e.  a  combined  flexion  and  adduction,  as  in  crossing  the  legs.  It  also 
rotates  outwards.  The  Adductor  longus,  and  probably  the  Adductor  brevis 
(obturator  nerve),  have  the  same  action,  but  the  flexion  is  less  than  by  the 
pectineus.  The  Adductor  magnus  (obturator  and  great  sciatic)  causes  a  similar 
adduction,  but  while  its  upper  fibres  rotate  outwards  its  lower  fibres  rotate  in, 
and  are  employed  in  keeping  the  foot  straight  during  adduction  in  riding. 
This  is  very  difficult  if  these  fibres  are  paralysed.  The  foot  then  turns  out 
when  the  hip  is  flexed,  either  in  the  recumbent  posture  or  in  walking,  from  the 
preponderance  of  rotation  out  by  the  other  adductors.  When  all  the  adductors 
are  paralysed,  not  only  is  adduction  lost,  but  in  flexion  of  the  hip  the  foot  is 
moved  forwards  and  outwards,  instead  of  directly  forwards,  showing  that  there 
is  normally  a  synergic  action  of  the  abductors  and  adductors  with  the  flexors 
in  this  movement. 

Muscles  moving  the  Knee. — Extensors :  Rectus,  Vasti,  and  Crureus, 
together  called  the  E.  quadriceps  (ant.  crural  nerve,  L.  3  and  4). — The  vasti  act 
solely  on  the  knee-joint;  the  rectus  also  aids  in  flexing  the  hip,  but  chiefly 
when  the  knee  is  bent.  In  consequence  of  its  passage  over  the  hip-joint,  moi'e- 
over,  the  force  with  which  it  extends  the  knee  is  increased  by  the  simultaneous 
extension  of  the  hip.  This  efHect  is  useful  in  the  propulsion  forwards  of  the 
body  in  walking.     The  crureus  is  unimportant. 

In  paralysis  of  the  extensors  of  the  knee,  standing  is  still  possible  if  the  knee 
is  extended,  since  the  arrangement  of  the  articulation  renders  a  contraction  of 
the  extensors  unnecessary.  But  secondary  shortening  of  the  flexors  is  apt  to 
occur,  and  then  standing  becomes  impossible  because  the  knee  cannot  be  perfectly 
extended.  In  the  same  way,  walking  is  possible  if  the  leg  is  not  moved  forward 
beyond  the  vertical  position  ;  if  it  is,  the  knee  becomes  flexed  by  the  weight  of 
the  leg  and  foot,  and  the  patient  falls  when  he  attempts  to  rest  upon  it. 
Rising  from  the  kneeling  posture  in  the  ordinary  way  is  impossible.  In  partial 
paralysis  of  the  muscles,  as  in  pseudo-hypertrophic  paralysis  (q.  v.),  the  exten- 
sion of  the  knee,  in  rising,  is  facilitated  by  placing  the  hand  upon  it,  and 
so  bringing  the  centre  of  gravity  of  the  body  near  the  fulcrum  of  the  lever 
formed  by  the  femur.  If  the  vastus  internus  and  rectus  are  paralysed,  the 
vastus  externus  may  dislocate  the  patella  by  the  obliquity  of  its  traction.  The 
vastus  intei'nus  never  does  so  in  the  opposite  condition,  because  its  action  is  less 
oblique. 

Flexors  of  the  Knee. — These  are  all  supplied  from  the  same  spinal  roots, 
although  through  various  nerves — L.  4  and  5,  S.  1. — The  Sartorius  (ant. 
crural  nerve)  flexes  the  hip-  and  knee-joints,  and  has  a  feeble  power  of 
rotating  the  thigh  outwards  and  the  knee  inwards.  It  is  a  muscle  of  small 
importance. 

The  Gracilis  (obturator  nerve)  adducts  the  thigh  more  powerfully  than  it 
flexes  the  knee.     It  rotates  the  leg  inwards. 

The  Semitendinosus,  Biceps,  and  Semimerribranosus  (great  sciatic  nerve) 
are  not  only  flexors  of  the  knee  but  extensors  of  the  hip-joint,  and  are  the 
muscles  that  extend  the  hip  during  ordinary  walking,  the  gluteus  maximus 
(q.  V.)  being  called  into  action  only  during  special  efforts.  The  leg  is  rotated 
inwards  liv  the  semitendinosus,  outwards  by  the  biceps. 

In  paralysis  of  the  flexors  the  resulting  loss  of  the  power  ■)£  flexion  interferes 


48 


ACTION   AND   PARALYSIS   OF   MUSCLES. 


with  walking,  since  the  knee-joint  cannot  be  bent,  in  the  forward  movement  of 
the  leg,  until  the  thigh  is  flexed  sufficiently  to  permit  the  weight  of  the  foot  to 
flex  the  knee.  To  prevent  the  toes  striking  the  ground  the  foot  is  unduly  flexed 
on  the  leg.  The  loss  of  the  support  that  the  flexor  tendons  give  to  the  knee- 
joint  leads  to  an  undue  strain  on  the  ligaments,  which  become  stretched,  and 
slight  retroflexion  of  the  joint  may  occur. 

In  paralysis  of  the  muscles  that  extend  the  hip,  there  is  a  tendency  to  fall 
forwards  in  walking.  To  counteract  this  the  trunk  is  carried  backwards,  and  a 
fatiguing  strain  on  the  flexors  of  the  hip  results. 

In  paralysis  of  the  biceps,  the  leg,  during  flexion,  is  rotated  inwai'ds ;  when 
the  biceps  remains  and  the  other  muscles  are  paralysed,  there  is  an  undue  rota- 
tion outwai'ds.     The  effect  of  these  abnormal  movements  on  the  ligaments  of 


Tibialis  anticus, 
Extensor  digit 


Peroneus  brevi 


Extensor  long,  po': 


Dorsal  interosse 


Ext.  poplit.  n. 


Gastrocnem.  (outer  head). 
Peroneus  longus. 


Soleus. 


Flexor  long.  poUicis. 


Extensor  brev.  digit. 


Abductor  minim,  digit. 


Pia.  26. — Motor  points  of  leg,  outer  side  (Erb). 


the  joint  is  such  that,  after  a  time,  the  amount  of  rotation  becomes  greater  than 
is  possible  in  health. 

The  Popliteus  (internal  popliteal  nerve)  has  but  a  feeble  power  of  flexing  the 
knee.  Its  chief  action  is  to  rotate  the  leg  inwards  when  the  knee-joint  has  been 
flexed. 

Muscles  movhto  the  Foot. — Extensors  of  Foot  on  Leg* — The  Gastro- 
cnemius and  Soleus  (internal  popliteal  branch  of  the  sciatic — L.  5  and  S.  1) 

•  These  muscles  are  sometimes  termed  "  plantar  flexors,**  because  they  are  homo* 
logons  with  the  flexors  of  the  wrist.    The  term  is  a  bad  one,  since  it  involves  a  nse 


MUSCLES    OP    LOWER   LIMB. 


49 


have  the  same  action.  They  extend  the  hinder  part  of  the  foot  and  draw  down 
the  outer  side  of  the  forepart  of  the  foot,  but  very  little  the  inner  side.  Hence 
the  foot  is  rotated,  so  that  the  dorsum  looks  outwards,  while  the  whole  foot  is 


Sciatic  n.      ^i 

Biceps  (loug  head) 
Biceps  (short  head) 


Sxt.  poplit.  n. 
Gastrocnem.  (ext.  head) 

Soleus 
Flexor  long.  poll. 


Gluteus  maximus. 


*        Adductor  magnus. 
Seniiteiidinosus. 
Seiidmembranosus, 


Int.  poplii.  n. 

Gastrocnem.  (int.  head). 
Soleus. 

Flexor  digit,  comm. 
Tibial  n. 


Fig-.  27. — Motor  points,  back  of  thigh  and  leg  (Erb). 

turned  inwards  on  the  axis  of  the  leg.  The  peculiar  inversion  and  adduction 
that  thus  accompanies  extension  is  due  to  the  form  of  the  articular  surfaces. 
The  gastrocnemius  has  very  little  power  of  flexing  the  knee,  but  the  extension 

of  the  word  •'  flexor  "  in  absolute  contradiction  to  its  proper  signification.  We 
ought  not  to  frame  a  descriptive  term  such  as  this  on  an  analogy  which  involves  a 
contradiction  to  the  description.  Flexion  is  bending,  a  movement  from  a  straight 
line;  and  extension  is  less  bending,  a  movement  towards  a  straight  (stretched, 
extended)  line.  To  call  a  movement  townrd  a  straight  line  "  flexion,"  because  in  the 
arm  the  similar  movement  produces  this  effect,  is  a  process  that  is  not  description, 
but  the  statement  of  an  analogy — the  worst  possible  kind  of  nomenclature.  Names 
should  be  descriptive  or  they  should  be  arbitrary.  Neither  theory,  nor  analogy, 
nor  homology  should  have  any  part  in  them.  What  we  call  homology  is  merely  a 
species  of  analogy;  it  rests  on  inference  and  reasoning,  not  on  simple  absolute 
aspect.  Nothing  that  rests  on  reasoning  is  inherently  stable  and  free  from  change. 
VOL.  I.  4 


50  ACTION  AND  PARALYSIS    OF    MUSCLES. 

of  the  knee  increases  the  effect  of  the  muscle  on  the  ankle-joint,  especially  in 
walking,  just  as  we  have  seen  that  the  extension  of  the  hip  augments  the  force 
with  which  the  contracting  rectus  extends  the  knee.  For  direct  extension  of  the 
ankle,  the  peroneus  concurs  and  opposes  the  inversion.  The  only  diffei'ence 
between  the  gastrocnemius  and  soleus  is  that  the  latter,  having  no  attachment 
to  the  femur,  can  extend  the  ankle  when  the  knee  is  flexed  as  well  as  when  it  is 
extended.  In  paralysis  of  these  muscles,  extension  of  the  ankle  (by  the 
peroneus  longus  and  flexor  longus  digitorum)  is  extremely  feeble,  and  the  foot 
can  scarcely  be  carried  beyond  a  right  angle.  Walking  is  greatly  interfered 
with ;  standing  on  tiptoe  is  impossible.  The  unopposed  peroneus  longus 
causes  eversion  of  the  foot,  lowers  the  head  of  the  first  metatarsal  bone,  and 
deepens  the  plantar  ai'ch.  In  time  the  ankle-joint  becomes  over-flexed,  the  heel 
considerably  lowered,  and  the  plantar  muscles  and  fascia  become  shortened. 
The  resulting  deformity  is  termed  talipes  calcaneus  (Fig.  28). 


'A 


Fia.  28. — Talipes  calcaneus  from  atrophic  paralysis  of  the  calf  muscles,  with 
flexion  of  tlie  middle  and  distal  phalanges  of  the  toes,  from  paralysis  of 
the  interossei. 

The  'p&roneus  longus  (musculo-cutaneous  nerve,  from  external  popliteal  of 
sciatic — S.  1  and  2)  everts  the  foot,  lowering  the  inner  border,  narrowing  the 
foot,  and  increasing  the  plantar  arch.  It  also  turns  the  whole  foot  out  on  the 
axis  of  the  leg.  It  has  a  very  feeble  power  of  extending  the  ankle.  It  keeps 
down  the  inner  part  of  the  foot  during  extension  by  the  calf  muscles,  as  in 
walking.  In  paralysis  of  this  muscle  the  inner  part  of  the  front  foot  is  not 
supported  during  extension,  and  yields  to  slight  force.  The  foot  becomes 
adducted  and  rotated,  so  that  the  sole  is  directed  inwards,  in  consequence  of  the 
unopposed  action  of  the  sural  muscles.  The  inability  to  press  the  inner  part  of 
the  ball  of  the  foot  firmly  against  the  ground  leads  to  over-action  of  the  flexors 
of  the  great  toe.     The  plantar  arch  is  lessened  ;  there  is  "  flat-foot." 

Flexors  of  the  Foot. — (Both  are  supplied  by  the  anterior  tibial  bi'anch  of  the 
external  popliteal  nerve — L.  5  and  S.  1.)  The  Tibialis  anticus  produces  simul- 
taneously three  movements :  it  elevates  the  inner  part  of  the  front  foot  (oppos- 
ing the  peroneus  longus)  ;  it  flexes  the  ankle-joint,  and  addncts  the  foot.  The 
Extensor  longus  digitorum,  besides  extending  the  toes,  flexes  and  abducts  the 
foot.  The  abduction  is  in  consequence  of  the  outward  position  of  its  tendons 
beneath  the  annular  ligament.  These  two  muscles  together  produce  direct 
flexion  of  the  foot,  or  flexion  witli  adduction  or  abduction,  as  the  force  of  one  or 
the  other  preponderates.  Paralysis  of  either  weakens  flexion,  and  the  corre- 
sponding lateral  movement  is  lost,  flexion  being  accompanied  by  the  deviation 
effected  by  the  muscle  that  remains.  The  defect  in  flexion  is  greatest  in  para- 
lysis of   the   tibialis,  and  the  loss   of  the    instinctive    flexion,   when   the   leg 


MUSCLES  OP   LOWES  LIMB. 


51 


ii  brought  forward  in  walking,  canses  the  foot  to  catch  against  the  gronnd. 
Paralysis  of  the  flexors  is  followed  by  seoondaiy  contraotuie  of  the  extensors, 


FlO.  29. — ^Talipes  equinns,  dae  to  atrophy  of  the  tibialis  anticus  and 
secondary  contracture  of  the  calf  muscles.  In  A  the  foot  is  shown  at 
rest ;  there  is  slight  equino-varus.  In  b  it  is  shown  during  flexion, 
and  the  vams  is  changed  to  valgus  by  the  action  of  the  peronens 
longus.  Note  the  increased  extension  of  the  toes  in  B  from  the  com> 
pensatory  over-action  of  the  long  extensors  of  the  toes.  (After 
Duchenne.) 

•nd  talipes  equinus  results  (Fig.  29),  which  is  the  greater  the  longer  the 
palsy  has  lasted  (Fig.  30).    Its  occurrence  is  facilitated,  in  many  cases  of  palsy. 


Fio.  SO. 


Fia.  81. 


Fio.  30. — Extreme  talipes  eqainns  from  old-standing  palsy  of  the  tibialis 
anticus  (infantile  paralysis)  and  extreme  contraction  of  the  calf  muscles.  No 
flexor  movement  was  possible. 

Fig.  31. — Paralysis  of  the  interossei  and  the  adductor  and  short  flexor  of  the 
great  toe.  The  first  phalanges  are  over-extended  and  the  second  are  flexed, 
while  the  hollow  of  the  sole  is  increased.     (After  Duchenne.) 

by  lessened  growth  of  the  bones  of  the  leg,  so  that  the  ball  of  the  foot  only 
touches  the  ground  when  the  foot  is  extended.  There  is  usually  slight  rotation 
inwards  of  the  foot  at  rest,  even  when  the  tibialis  is  paralysed  (see  Fig.  29,  a), 
because  such  rotation  is  produced  by  the  sural  extensors  (p.  42) ;  but  in  this  case 
the  slight  valgus  at  rest  is  changed  to  varus  on  an  attempt  to  flex  the  ankle 
(Fig.  29,  B). 


52  ACTION   AND    PARALYSIS    OP   MUSCLES. 

The  Peroneus  brevis  (musculocutaneous  [peroneal]  branch  of  ext.  popliteaJ 
nerve — S.  1  and  2)  abducts  the  foot  and  rotates  it,  raising  the  outer  edge. 

The  Tibialis  posticus  (posterior  tibial  nerve  from  int.  pop. — L.  5  and  S.  1) 
adducts  the  foot  and  curves  it,  rendering  the  outer  border  and  instep  more 
convex,  Its  power  of  adduction  is  greater  than  that  of  the  tibialis  anticus,  and 
it  does  not  rotate  the  foot  in  the  same  manner. 

These  two  muscles  alone  have  the  power  of  adducting  and  abducting  without 
flexing  or  extending ;  and  in  their  paralysis  these  simple  movements  are  lost.  If 
one  only  is  paralysed,  a  deformity  develops  corresponding  to  the  action  of  the 
other  muscle, — talipes  valgus  in  paralysis  of  the  tibialis  posticus ;  t.  varus  in 
that  of  the  peroneus  brevis. 

The  muscles  moving  the  toes  present,  in  their  mode  of  action,  a  close  corre- 
spondence to  those  of  the  fingers. 

The  Extensor  longus  digitorum  and  the  Extensor  longus  pollicis  (anterior 
tibial  nerve— L.  5  and  S.  1)  extend  chiefly  the  first  phalanges,  while  the  Flexor 
longus  digitorum  and  Flexor  brevis  (postei-ior  tibial  nerve — S.  1  and  2)  flex  the 
last  two  phalanges.  The  Lumbricales  and  the  Interossei  (post,  tibial  nerve 
by  ext.  and  int.  plantar — S.  1  and  2),  together  with  the  Abductor  and  Flexor 
brevis  m,inimi  digiti,  oppose  both  the  other  extensor  and  flexor  muscles,  flexing 
the  first  phalanx  and  extending  the  others.  This  action  is  of  great  importance 
in  walking,  since  they  give  the  last  propulsion  to  the  body  as  the  ball  of  the 
foot  leaves  the  ground.  The  Abductor,  Adductor,  &vi^  Flexor  hrevis  jpollicis 
(plantar  nerves  fi-om  post,  tibial — S.  1  and  2j  have  a  similar  action  on  the 
great  toe,  but  with  adduction  or  abduction  respectively.  The  interossei  also 
produce  a  lateral  movement  of  the  toes,  but  this  action  is  of  little  practical 
importance.  In  paralysis  of  the  common  extensor  of  the  toes,  and  of  the 
proper  extensor  of  the  great  toe,  the  tonic  force  of  the  interossei  and  analogous 
muscles  produces  persistent  flexion  of  the  first  phalanges  and  extension  of  the 
others.  If  the  conditions  are  reversed,  and  the  latter  muscles  are  paralysed,  the 
first  phalanges  are  over-extended,  sometimes  even  subluxated,  and  the  two 
other  joints  are  flexed,  so  that  a  claw-like  form  of  foot  is  the  result  (Figs.  31 
and  28).  The  final  propulsion  in  walking,  above  described,  is  much  interfered 
with,  and  the  attempt  is  painful  because  the  ends  of  the  toes  are  turned  towai'ds 
the  ground. 

Other  examples  of  the  effects  of  paralysis  of  the  muscles  of  the  arm 
and  leg  are  given  in  the  illustrations  to  the  chapter  on  Infantile 
Paralysis  (Acute  Polio-mjelitis^. 


53 


THE  GENEEAL  CONSTITUTION  OF  THE  NEEVOUS 

SYSTEM* 

Our  conceptions  of  the  elementary  arrangement  of  the  nen  oua 
system  have  been  changed  during  the  last  nine  years  by  important 
histological  discoveries,  which  clear  many  obscurities,  give  new  signi- 
ficance to  facts  before  imperfectly  perceived,  and  involve  new  patho- 
logical conceptions,  general  and  special.  Although  new  difficulties  have 
arisen,  as  they  must  do  at  each  step  forward,  numerous  facts,  which 
before  were  mysterious,  become  intelligible,  and  their  elucidation  con- 
stitutes confirmation  of  the  truth  of  the  discoveries.  The  evidence 
has  been  received  as  adequate  by  aU  physiologists.  Pathologists 
must  therefore  accept  them,  and  reconstruct  their  conceptions.  It  is 
accordingly  necessary  to  give  an  outline  of  the  constitution  of  the 
cerebro-spinal  nervous  system  as  at  present  discerned,  even  of  that 
which  is  not  of  direct  present  application  to  pathology,  but  which  may 
become  so  and  is  essential  for  the  perception  of  the  whole.  The  facts 
have  been  found  also  to  be  true  of  the  sympathetic  system.f 

These  discoveries  are  the  result  of  a  method  of  metallic  staining, 
first  devised  in  detail  by  G-olgi  of  Pavia,  in  which  silver  is  reduced  in 
the  structures  that  have  been  impregnated  with  chromiimi  during 
hardening,  and  usually  also  acted  on  by  osmic  acid.  It  displays  the 
minute  structure  of  the  grey  matter  with  a  distinctness  and  in  a 
manner  that  reveal  facts  before  altogether  unseen. 

Almost  the  first  definite  steps  were  the  results  obtained  in  the  Inver- 
tebrata  by  Nansen,  before  he  turned  his  face  from  polar  cells  to  Polar 
seas.  But  the  first  important  disclosures,  which  involved  the  radical 
<5hange  in  conception,  were  due  to  Ramon  y  Cajal  of  Barcelona  J  and  to 
the  veteran  v.  K6lliker,§  supplemented  and  diffused  by  Waldeyer.|| 
Since  1890  there  has  been  incessant  work  at  the  subject,  and  y  Cajal 
has  presented  to  us  the  chief  facts  that  had  then  been  ascertained,  in 
the  Croonian  Lecture  to  the  Eoyal  Society  in  1895. 

All  nerve-fibres  are  prolonged  processes  of  nerve-cells.  They  consist 
of  an  axis,  with  a  "  medullary  sheath  "  around  it  when  the  course  is 
long,  either  within  or  outside  the  central  organs.     Most  cells  have  but 

•  The  interpolation  of  this  account  of  the  recent  revolution  in  fundamental 
elements  of  our  knowledge,  although  not  quite  consistent  with  tlie  plan  of  the  book, 
«eeras  the  most  convenient  way  of  presenting  the  facts  to  the  reader.  They  pass 
below,  and  rise  above,  the  range  of  practical  knowledge  which  can  he  used  in  common 
work,  and  yet  are  changing  our  physiological  and  pathological  conceptions  in  a  manner 
*nd  degree  which  must  be  adequately  recognised,  although  much  of  their  effect  is 
ttill  uncertain. 

t  V.  Kolliker,  "  Histol.  Mittheil.,"  •  Wurzburg,  Sitznngsb.,'  Nov.  23rd,  1889. 

X  Previously,  •  Internat.  Monatschr.  f.  Anat.,'  1890,  Bd.  vii. 

§  Loc.  cit. 

II  ♦  Berlin,  med.  Wochenschr.,'  1891,  No.  28. 


54  ACTION  AND  PAEALTSIS  OP  MUSCLES, 

one  such  medullated  process,  larger  tlian  tlie  otliers ;  some  hare  two. 
The  other  processes  are  short ;  thej  soon  divide  and  branch  withio 
the  grey  substance :  the  long  medullated  processes  also  at  last  end 
by  dividing  and  ramifying. 

The  conception  formerly  held  may  be  thus  stated.  Of  the  ultimate 
divisions  of  the  short,  quickly -branching  processes  in  the  grey  matter, 
some  had  been  thought  to  join  the  terminations  of  similar  processes 
from  other  cells,  either  neighbouring  cells  in  the  grey  matter,  or  the 
terminal  branches  of  medullated  nerve-fibres,  processes  of  cells  far 
away.  Thus,  of  the  motor  cells  of  the  spinal  cord,  some  of  the 
processes,  passing  backwards,  were  believed  to  join  those  of  sensory 
cells  in  the  posterior  cornua,  of  which  the  medullated  process  was  a 
fibre  of  the  posterior  root.  The  constituent  elements  of  the  nervous 
system  thus  formed,  by  branch  union,  one  continuous  complex  net- 
work, with  paths  for  the  nerve-impulses,  due  to  union  and  continuity 
of  the  cell-processes.  The  paths  in  actual  use  were  determined  not 
only  by  such  union,  but  also  by  differences  in  "  resistance "  among 
those  which  continuity  provided.  The  "  resistance,"  which  thus  per- 
mitted an  energetic  impulse  to  spread  more  widely  than  a  slighter 
one,  was  varied  in  degree  by  fxmctional  activity ;  it  was  diminished 
by  the  repetition  of  the  same  activity,  and  it  was  also  varied  myste- 
riously by  other  nerve-impulses  from  various  sources  It  might  thus 
be  increased  so  as  to  "inhibit"  action.  In  so  far  as  any  attempt 
was  made  to  conceive  its  seat,  it  was  thought  of  as  in  the  cells,  or  in 
the  feltwork  of  uniting  processes  in  the  grey  substance,  called  "  spongy,** 
from  its  blending  trabeculse. 

Much  of  this  conception  is  retained.  But  the  more  the  methods  of 
examination  were  improved  actual  luiion  of  the  branches  of  cell-processes 
became  less  perceptible  under  the  microscope.  The  development  of  the 
use  of  staiaing  agents,  especially  metallic,  has  increased  the  power  of 
discrimination.  Yariety  of  aspect  can  be  thus  produced  in  structural 
elements  that  before  were  indistinguishable.  By  the  use  of  such 
methods,  and  especially  that  of  Golgi,  the  branches  of  the  processes 
and  fibres  have  been  clearly  traced,  and  they  have  been  found  not  to 
unite.  They  are  distinctly  seen  to  end  in  the  structural  material  ia 
which  the  nerve-cells  lie,  sometimes  by  an  enlarged  knob-like  extremity, 
sometimes  by  a  poiat.  Often  the  branches  cross  and  even  interlace, 
with  the  semblance  of  union,  but  close  observation  shows  that  it  is  a 
semblance  only.  Discontinuity  is  found  to  be  the  general  rule. 
Whether  invariable  or  not  is  still  undecided;  it  is  most  difficult  to 
exclude  union  in  the  dense  felt  of  fibrils  which  the  branching  processes 
form  in  some  parts,  the  "  neuro-pUema  "  of  His,  and,  moreover,  the 
general  rule  of  free  endings  is  compatible  with  occasional  actual 
union.* 

From  this  it  follows  that  the  "  nervous  system  *'  consists  of  discon* 

•  Cf.  Masius,  'Arch,  de  BioU'  1892;  Fritsch,  •  Brit.  Assoc.  Report,'  1892. 


GENERAL    CONSTITUTION    OP  THE    NERVOUS   SYSTEM.         55 

tinuous  elements,  each  a  cell-body  with  its  processes,  long  and  short. 
For  these  elements  the  name  "neuron,"  proposed  by  Waldeyer,*  has 
been  all  but  universally  adopted ;  for  distinctiveness,  its  plural  is 
formed  according  to  the  living  language,  and  not  the  classical  form — 
in  English  it  is  "  neurons,"  in  German,  "  neuronen,"  in  French, 
*'  neurones."  The  chief  process,  the  medullated,  or  axis-cylinder  process, 
is  called  the  "  axon,"  or  "  axis-process." f  The  branching  processes  in 
the  gi*ey  matter  are  termed  the  "  dendrons,"  the  branches  of  these 
"  dendrites."  t 

From  the  axons  there  often  spring  fine  fibres,  which  pass  off  at 
right  angles,  termed  "  collaterals  "  by  Ramon  y  Gajal,  "the  relations  of 
which  are  imperfectly  known.  They  have  been  seen  to  end  by  a  T-like 
division  and  ultimate  branching. 

The  definite  and  extended  discernment  of  another  fact  deepens  the 
importance  and  application  of  that  just  described. 

Thirty  years  ago  §  Max  Schultze  discovered  and  depicted  the 
fact  that  the  axis-cylinder  is  compound  and  not  simple,  that  it 
consists  of  a  large  number  of  fibrils, — "  primitive  fibrils,"  he  termed 
them.  He  pointed  out  that  they  could  be  traced  throughout  the 
fibre,  separated  by  a  finely  granular  substance.  They  could  be  well 
seen  where  the  axis-cylinder  widens  in  joining  the  nerve-cell,  that 
is,  where  the  cell  narrows  into  its  chief  process.  Others  could  not 
see  the  significant  striation,  among  them  Eanvier,  whose  work  domi- 
nated science  throughout  the  next  fifteen  years,  ||  and  the  fact  was 
practically  ignored.  The  axis-cylinder  was  regarded  as  simple  and 
integral,  \mtil  recent  methods  have  enabled  the  clear  establishment  of 
the  correctness  of  Max  Schultze's  observations.  The  niimber  of 
fibrils  that  constitute  an  axis-cylinder  is  considerable.  Obersteiner 
has  found  that  there  are  about  fifty  in  an  axis-cylinder  of  the  sciatic 
nerve  of  the  frog.  If 

*  Loc.  cit. 

t  Because  the  "  axon  "  is  the  separate  *'  nerve,"  wlien  one  exists,  an  attempt 
has  been  made  to  make  current  the  nse  of  "  neuron  "  for  this  alone.  But  etymo- 
logical consistency  has  little  influence  on  the  vitality  of  names.  The  use  of 
"neuron  "for  the  whole  element  has  become  so  general  th;it  lesistHnce  to  it  is  futile. 
Moreover,  the  conception  attached  to  it  in  use  is  already  definitely  detached  from 
its  etymology.  Lastly,  although  the  cell-body  and  its  processes  are  one,  to  have 
only  the  word  "  cell "  for  the  whole  element,  a  word  that  will  still,  inevitably,  be 
applied  to  the  cell-body,  leaves  the  latter  without  nominal  distinction  from  the 
other  two  pans  of  the  element — the  neuron  and  the  dendrons.  Hence  the  word 
"  neuron  "  is  here  used  in  the  established  senses. 

X  "Dendrite"  has  also  been  used  as  equivalent  to  "dendron,"  and  still  is;  but  the 
need  for  distinguishing  the  twi^rs  from  the  process  itself  makes  it  highly  probable 
that  this  convenient  separation  of  the  words  will  become  universal. 

§  In  1868 ;  see  Strieker's  *  Histology '  (New  Syd.  Soc.  trans.).  The  obtervationa 
were  confirmed  by  Babuchin  in  1868  and  1869. 

II  See  further  on  Structure  of  the  Nerves,  p.  62. 

•JT  Personal  communication. 


56  ACTION  AND  PARALYSIS   OF  MUSCLES. 

These  fibrils  consist  of  a  conducting  substance,  tbe  "  hyaloplasm " 
of  Leydig  and  Nansen  (so  termed  from  its  greater  translucency  in 
hardened  tissue).  This  is  isolated  by  a  slightly  granular  material, 
distinguishable  chiefly  by  its  difference  of  aspect  and  staining  after 
the  changes  produced  by  hardening  agents.  It  has  been  termed  the 
"  spongioplasm  " — "  plasm  "  because  it  also  has  a  soft  consistence, 
"  spongio  "  because  it  is  said  to  form  trabeculae,  which  constitute  the 
chief  element  in  the  groimd  substance,  or  matrix,  of  the  "  spongy  " 
grey  substance*  in  which  the  cells  lie  and  their  processes  branch. 

From  this  compound  constitution  of  the  axis,  we  must  infer  that  its 
terminal  division,  into  branches  is  merely  the  separation  of  these  fibrils, 
first  into  groups,  and  at  last  into  single  fibrils — "  primitive  fibrils."  It 
is  so  at  the  periphery  and  in  the  grey  matter.  The  final  twigs  may 
be  termed  "  axites,"  if  a  special  designation  seems  needed.f 

The  short  branching  processes  have  been  found  to  consist  of  similar 
fibrils,  and  this  is  true  also  of  those  supposed  to  be  extensions  of  the 
substance  of  the  cell,  and  therefore  called  "  protoplasmic  processes." 
This  name  should  be  given  up  with  the  conception  attached  to  it. 
The  opinion  that  their  function  is  to  convey  nutritive  material  to  the 
cell-body  was,  indeed,  never  more  than  an  hypothesis,  resting  on  no  real 
foundation.  J 

With  the  recognition  of  the  fibrillary  constitution  of  the  axon  and 
dendrons,  has  been  associated  an  equally  important  recognition  of 
the  fact  (most  clearly  depicted  by  Max  Schultze  §)  that  their  fibrils 
pass  through  the  body  of  the  nerve-cell  without  interruption.  Those 
of  the  axon  diverge  to  pass  to  the  several  dendrons,  but  those  of 
the  latter  do  not  pass  all  to  the  axon.  Some  pass  directly  to  other 
dendrons,  so  that,  in  such,  there  must  be  conduction  from  the  cell 
as  well  as  towards  it.||  Moreover,  both  y  Cajal  and  Kolliker  have 
observed,  in  certain  cells  of  the  cerebellum,  all  the  fibrils  of  an 
axis-cylinder  pass  directly  to  a  dendron,  scarcely  entering  the  body  of 
the  cell. 

Twigs  from  such  processes  of  small  cells,  especially  in  the  Inver- 
tebrata,  have  been  said  to  join  the  elements  of  the  neuroglia.^     The 

•  Apathy,  'Biol.  Ceutialbl.,'  1889.  The  term  "spongy  grey  substance"  was 
before  in  use  on  account  of  the  sponge-like  interlacement  of  the  cell-processeB. 
The  conception  attached  to  it  has  thus  become  somewhat  confused. 

t  Especially  since  one  writer  has  termed  them  neurites,  which  would  be  confusing 
unless  the  axis-proceps  were  termed  the  "  neuron,"  as  it  certainly  will  not  be. 
The  only  projier  use  of  this  word  (neurites  or  neuronites)  would  be  for  the  terminal 
twig.s  in  general. 

J  That  these  dividing  branches,  long,  and  not  obviously  permeable,  should  be  the 
channels  by  which  the  cell  receives  that  which  could  pass  directly  through  its  walls, 
was  simply  a  positive  inference  from  the  negative  fact  that  no  other  function  was 
obvious.  That  we  must  cease  to  consider  these  processes  "  protoplasmic**  and 
nutritional  was  insisted  on  in  1890  by  Rabl  Ruckhardt  ('  Neurolog.  Centralb.'). 

§  Loc.  cit. 

(I  V.  KoUiker,  '  Wuitzburg.  Sitzungsb.,'  Nov.  23rd,  1889. 

^  Among  others  by  Ladowsky, '  Verhandl.  Med.  Congress,'  Berlin,  1890,  ii,  92. 


GENERAL  CONSTITUTION  OF  THE    NERVOUS    SYSTEM.  67 

fact  is  in  harmony  with  the  origin  of  both  from  the  same  embryonal 
tissue.  The  differentiation  of  the  nerve-elements  from  this,  may  leave 
some  blending  of  structure.  It  must  be  remembered  that  there  is 
much  uncertainty  regarding  the  natiire  of  many  of  the  smaller  cells, 
and  it  is  conceivable  that  some  are  nervous  and  some  neuroglial,  and 
that  there  may  not  be  always  actual  separation  of  their  connections. 

An  essential  difference  between  the  axon  and  the  dendrons  cannot 
as  yet  be  established.  Branching  does  not  differentiate  them,  for  the 
axon  also  branches  after  a  longer  course.  Nor  does  either  multiplicity 
or  length.  The  cells  of  the  posterior  ganglia  of  the  cord  have  but  one 
axon  and  one  dendron,  united  for  a  short  distance  as  the  single  process 
of  the  "  unipolar  "  cell.  These  two  processes  are  of  nearly  equal  length 
in  the  case  of  those  cells  that  give  rise  to  the  fibres  of  the  posterior 
median  column ;  these,  reaching  the  cord  by  the  posterior  roots,  ascend 
to  the  level  of  the  medulla,  while  the  other  division  of  this  cell-process 
probably  comes  from  a  muscle  as  far  away.  We  see  also,  La  this 
instance,  the  absence  of  any  real  distinction  from  the  direction  of  con- 
duction. Although  the  axons  of  the  motor  cells  conduct  from  these, 
so  also  must  some  of  those  dendrons  to  which  fibrils  pass  from  others. 
Moreover,  in  the  case  of  the  posterior  root-fibres  which  go  to  the  local 
grey  matter,  the  long  fibre  from  the  periphery  conducts  towards  the 
cell  ;  the  shorter  fibre,  which  soon  branches  in  grey  substance,  con- 
ducts from  it.  Opinions  may  differ  as  to  which  should  be  regarded  as 
axon  and  which  as  dendron,  but  analogy  suggests  that  the  latter  should 
be  that  which  soon  ends  in  the  grey  substance.  But  it  is  difficult  to 
avoid  regarding  each  fibre  of  the  cells  related  to  the  posterior  median 
column  as  an  axon.  There  is  no  reason  for  regarding  either  a  dendron 
or  an  axon  as  an  essential  element  of  a  cell.  Yet  the  distinction  is 
necessary,  and  the  general  application  of  the  names  is  justified  by 
the  common  difference  between  the  one  medullated  process,  which 
passes  out  of  the  grey  matter,  and  the  other  numerous  processes,  which 
are  not  medvdlated,  and  soon  divide  within  the  grey  substance. 

The  extent  to  which  the  physiology  of  the  nervous  system  is  inferred 
from  its  minute  anatomy,  is  illustrated  by  the  revolution  in  our  con- 
ceptions produced  by  the  discovery  of  the  discontinuity  of  its  elements, 
the  fibrillary  constitution  of  the  conducting  structures,  and  the  course 
of  the  fibrils.  These,  passing  through  the  nerve-cells,  without  inter- 
ruption, can  merely  conduct  through  the  cell-body  as  they  do 
elsewhere  in  their  coui'se.  With  this  fact  disappears  the  old  idea,  so 
simple,  apparently  so  adequate  and  reasonable  from  its  superficial 
analogies — the  idea  that  the  nerve-cells  are  the  seat  of  the  production 
of  nerve-impulses,  that  in  them  "  nerve  force  "  is  generated  from  the 
latent  energy  stored  in  the  nutritional  compounds  which  have  entered 
into  their  constitution.  But  we  cannot  have  such  generation  where 
the  fibrils  are  continuous.  For  it,  we  must  look  to  their  extremities, 
formerly  supposed  to  be  in  the  cell-body,  now  perceived  to  be  in  the 


58  ACTION   AND  PARALYSIS  OP  MUSCLES. 

"  spongy  grey  substance."  TKe  process  must  be  wliere  the  continuous 
fibrils  begin  in  the  centre,  as  it  is  in  the  skin  and  other  parts  of  the 
periphery,  in  the  case  of  fibrils  that  conduct  thence.  As  all  the  nerve- 
impulses  that  reach  the  centres,  through  which  external  influences  act, 
and  the  environment  is  perceived,  originate  in  the  minutely  separate 
nerve  material  at  the  extremity  of  the  afferent  fibrils,  so  all  the  out- 
going impulses,  and  those  which  pass  from  one  part  of  the  centres  to 
another,  originate  in  their  special  form  at  the  analogous  extremities  of 
the  fibrils  in  the  grey  substance.  We  must  conceive  these  impidses 
arising  in  the  extremities  of  the  "  cellulipetal "  dendrites  (to  use  the 
somewhat  cumbersome  term  of  v.  KoUiker),  being  excited  in  some 
way  by  the  stimulus  of  the  impulses  which  reach  the  contiguous 
extremities  of  other  "  cellulifugal "  fibrils,  dendrites  or  branches  of 
the  axon  of  a  distant  cell.  The  incoming  impulses  may  excite  those 
that  go  out,  as  impulses  are  excited  at  the  periphery  by  other 
forms  of  energy  ;  or  there  may  be  a  process  of  conduction,  through 
the  intervening  ground-substance, — the  matrix  in  which  the  cells  lie 
and  the  dendrites  end.  For  the  increase  in  the  amount  of  nerve- 
energy  which  often  takes  place,  there  must  be  more  than  conduction, 
its  production  must  be  "  excited  "  by  that  which  is  conducted.  We  can 
conceive  such  excitation  without  continuity,  especially  when  we  con- 
sider that  the  stimulation  of  muscular  protoplasm  is  by  the  impulses 
in  nerves  which  terminate  on  the  fibres,  and  not  in  continuity  with 
the  contractile  substance — indeed,  separated  from  it  by  a  structure 
apparently  quite  different  in  its  simpler  nature. 

If  this  conception  of  the  origin  of  the  impulses  in  the  terminations 
of  the  fibrils  seems  less  easy  than  their  origin  in  the  more  massive 
nerve-cells,  we  must  remember  how  multiphcity  neutralises  minuteness. 
Indeed,  the  minuteness  of  the  conducting  fibrils  almost  involves  a 
multiplicity  of  minute  sources  of  nerve-impulses, — ^which  must,  more- 
over, be  far  from  minute  in  comparison  with  the  molecules,  from  the 
latent  energy  of  which  the  impulses  proceed.  All  modem  discoveries 
prepare  us  for  finding  every  mass  to  be  an  aggregation,  and  each 
perceptible  quantity  of  energy  to  be  made  up  of  minute  constituents, 
every  one  of  which  is  definite,  both  in  its  separate  character  and  in 
its  contribution  to  the  effect  of  the  whole.  The  finely  divided  nerve- 
substance  will  constitute  a  total  source  of  energy,  as  adequate  in  extent 
as  a  nerve- cell,  with  a  more  obvious  facihty  for  the  renewal  of  the 
molecules  lost  in  functional  action,  and  far  greater  opportunity  for 
varying  relation  to  the  structures  from  which  the  impulses  are 
received. 

What,  then,  is  the  function  of  the  nerve-cell  ?  The  one  certain  fact, 
established  by  evidence  that  is  secure,  is  that  on  it  depends  the  life  of 
the  nerve-fibres — the  vitality  of  all  the  processes,  equally  that  of  the 
dendrons  and  of  the  axon.  The  evidence  of  this,  the  immediate  degene- 
ration of  any  separated  part,  is  well  known.  It  is  less  conspicuous  in 
the  case  of  the  dendrons,  but  sufficiently  certain.     How  the  influence 


GKJMEliAL   CONSTITUTION    OF    TH  K    NKRVOUS    SYSTEM.  59 

is  exerted  we  can  only  snrmise.  The  nucleus  of  every  cell  is  its  life 
centre,  and  in  some  way  the  nutrition  of  the  protoplasm  is  determined 
by  it.  The  cell-body  has  a  complex  structure,  dimly  perceptible  by 
the  help  of  reagents,  at  present  beyond  our  practical  comprehension. 
One  distinct  fact,  however,  is  that  the  protoplasm  of  the  cell,  extend- 
ing into  the  narrowing  axon,  thins  away  between  the  outer  sheath  and 
the  white  substance,  becoming  unrecognisable  until  one  of  the  many 
nuclei  is  reached.  Each  of  these  is  surrounded  by  like  protoplasm,  also 
thinning  of£  along  the  fibre  until  invisible.  It  is  certain  also  that  if 
the  cell-body  and  nucleus  are  destroyed,  or  the  fibre  separated,  the  pro- 
cess of  degeneration  begins  by  changes  in  these  nuclei.  They  seem  to 
be  concerned  in  carrying  on  the  influence  of  the  distant  cell,  and  it  is 
not  easy  to  conceive  any  other  path  for  this  influence  than  a  thin 
layer  of  protoplasm,  within  the  sheath,  continuous  from  nucleus  to 
nucleus,  vitally  influenced  by  these,  and  conveying,  by  its  own  nutri- 
tional state,  that  of  the  cell.  A  space,  in  this  position,  is  rendered 
visible  by  reagents  which  make  the  contents  of  the  sheath  shrink. 
Indeed,  coagulated  material,  in  small  quantity,  can  be  seen  within 
it.*  This  has  been  regarded  as  evidence  of  the  presence  during 
life  of  such  a  space  as  is  here  assumed  to  contain  connecting  proto- 
plasm. 

Many  facts,  previously  obscure,  become  more  intelligible  in  the  light 
of  the  new  discoveries.  The  knowledge  that  the  cell  governs  the 
nutrition  of  the  fibi-e,  and  the  belief  that  from  it  proceeded  the  nerve- 
impulses,  involved  a  correspondence  in  the  direction  of  nutritional  in- 
fluence and  of  conduction,  and  it  was  found,  in  fact,  that  conduction  and 
degeneration  were  in  the  same  direction  as  a  rule — but  only  "  as  a 
rule."  It  was  untrue  of  the  sensory  nerves.  These,  coming  from  the 
cells  of  the  ganglia  on  the  posterior  spinal  roots,  degenerate  downwards, 
but  they  conduct  upwards — an  anomaly  which  had  to  be  simply  ignored. 
The  fact  is  now  seen  to  be  in  harmony  with  others. 

The  arrest  of  secondary  degeneration  in  the  grey  matter,  the  fact 
that  the  degeneration  of  the  fibre  which  enters  the  grey  matter  never 
passes  on  to  the  next  nerve-cell,  through  which  conducted  impulses 
must  pass,  was  mysterious  when  they  were  believed  to  be  in  continuity. 
The  discontinuity  explains  it  at  once.  The  degeneration  is  limited  to 
the  single  neuron. 

The  division  of  the  axis-cylinder  at  its  terminal  ramification, 
whether  that  of  the  sensory  nerve  in  the  skin  or  of  the  motor  nerve  in 
the  muscle,  was  scarcely  intelligible  when  the  axis-cylinder  was  regarded 
as  a  single  conducting  path  ;  it  is  so  when  we  perceive  that  the  apparent 
division  is  simply  the  separation  of  its  constituent  fibrils,  each  a  dis- 
tinct conducting  path.  This  fact  is,  moreover,  especially  important  in 
relation  to  the  minute  localisation  of  the  various  forms  of  sensation  in 

*  ScbiefEeidecker,  *  Arch,  f .  mik.  Anat,,'  xxx,  1887. 


60  ACTION  AND   PARALYSIS  OP  MUSCLES. 

the  skin.  The  sensory  nerve-fibres  themselves  are  not  numerous 
enough  to  subserve  this  localisation,  which  could  not  result  from  the 
division  of  an  axis-cylinder  that  conducted  as  a  whole. 

The  fact  that  the  special  form  of  energy  termed  a  "  nerve-impulse  ** 
may  arise  in  a  fibre  in  its  course  under  the  action  of  a  stimulus,  that 
the  capacity  of  the  fibres  is  not  limited  to  "  conduction,"  is  less 
surprising  if  we  conceive  all  such  impulses  as  produced  at  their 
extremities. 

While  old  difficulties  thus  disappear,  and  many  facts  become  more 
readily  intelligible,  so  great  a  change  of  our  conceptions  involves 
new  difficTolties.  Not  onJy  are  the  structures  concerned  most  minute, 
but  their  discernment  depends  on  the  use  of  reagents  which  reveal 
them  by  developing  differences  in  aspect  due  to  chemical  changes; 
new  physical  characters  have  been  produced  by  other  agents  of 
the  same  nature.  There  seems  but  little  original  difference  in  consist- 
ence between  the  material  that  constitutes  the  conducting  axis  of  the 
"  primitive  fibrils,"  and  that  of  the  separating  substance.  Both  are 
apparently  almost  diffluent  during  life,  and  are  indistinguishable 
physically  until  their  difference  in  constitution  is  revealed  by  the  action 
on  them,  first  of  the  chemical  agents  that  harden,  and  then  of  those 
that  stain.*  Both  processes  involve  molecular  chemical  changes,  which 
multiply  initial  differences,  and  leave  the  actual  amount  of  these  more 
obsciire  than  we  are  apt  to  think. 

The  conducting  element  of  the  fibrils,  the  "  hyaloplasm,"  or  "  kine- 
toplasm,"  is  said  to  be  more  diffluent  than  the  separating  "  spongio- 
plasm,"  but  the  difference  may  be  chiefly  due  to  the  hardening  agents 
used.  Continuous  within  the  tubules,  and  through  the  cell-body  into 
the  dendrons,  it  has  been  said  that  beyond  these  it  is  also  continuous 
with  similar  material  in  the  matrix  of  the  spongy  substance.  Into  this 
also  the  spongioplasm  is  said  to  pass  and  form  a  trabecular  structure. 
The  fact  that  the  fibrils,  as  stained  by  Golgi's  method,  apparently  end, 
must  indicate  that  these  elements,  if  continuous  with  those  of  the 
matrix,  are  different  in  nature  in  some  degree.  The  difference  may  be 
much  exaggerated  by  reagents,  and  yet  sufficient  to  constitute  the 
functional  discontinuity  which  is  generally  assumed  to  be  as  stmctur- 
ally  absolute  as  it  appears  in  the  sections. 

Until  we  know  what  the  structure  of  the  matrix  is,  we  can  scarcely 
conjecture  the  nature  of  the  process  that  occurs  in  it  between  the 
dendrites.     "  Conduction "  may  have  been  too  readily  regarded  as 

*  la  connection  with  the  fact  that  molecular  differences  between  substances 
apparently  the  same,  even  when  examined  with  the  utmost  minuteness,  may  be 
revealed  by  staining,  the  facts  of  the  colour  photography  of  Cassagne  may  be 
noted.  The  waves  of  light  which  differ  only  in  rapidity  and  length,  but  by  this 
excite  different  elements  of  the  retina,  reduce  a  special  silver  snlt  in  the  gelatine  film 
in  different  degree.  Extremely  slight  as  must  be  the  resulting  difference  in  the 
molecules  which  is  produced,  it  apparently  suflSces,  augmented  by  some  additional 
chemical  process,  to  make  each  of  them  retain  some  stains  and  not  others. 


GENERAL    CONSTITUTION    OF   THE    NERVOUS  SYSTEM.         61 

impossible,  and  it  is  not  clear  tliat  actual  conduction  of  nerve- impulses 
is  incompatible  with  the  augmentation  we  include  under  the  term 
"excitation."  An  increase  must  occur  when,  for  instance,  in  many 
reflex  actions  the  afferent  impulse  that  reaches  the  spinal  cord  is  minute 
in  proportion  to  that  which  leaves  the  centre.  We  seem  thus  to  have 
a  process  of  stimulation  comparable  to  that  in  the  cutaneous  nerves 
(themselves  apparently  analogous  to  dendrites),  under  the  influence  of 
some  other  form  of  energy  from  the  outside.  In  this  connection  it  is 
especially  noteworthy  that  many  dendrites  terminate  in  knob-like 
enlargements,  suggesting  receptive  functions. 

"We  do  not  know  the  nature  of  a  "nerve-impulse."  We  must, 
however,  assume  that  it  is  energy  in  some  form  of  motion,  released 
from  that  previously  latent  in  the  nerve- substance,  apparently  the 
•'  kinetoplasm  "  or  "  hyaloplasm."  Chemical  change  occurs  by  which 
simple  compounds  are  formed,  as  in  aU  similar  processes,  and  the 
simplest  conception  is  that  the  process  is  like  that  in  a  train  of 
explosive  material,  modified,  restricted,  but  perhaps  accelerated  by  the 
influence  of  life.* 

The  motion  of  a  touch,  which  acts  upon  the  nerve-endings  in  the 
skin,  reaches  them  by  passing  through  the  skin  as  the  simplest  form 
of  motion ;  so  also  pass  the  special  forms  of  wave  motion  that  con- 
stitute electricity  and  heat.  A  differentiation  of  the  nerve-tissue  in 
the  ending  seems  to  enable  their  molecules  to  receive  one  special  form 
of  motion  most  readily.  Hence  our  "  special  senses."  The  manner  in 
which  the  equilibrium  of  the  nerve- substance  is  disturbed  in  each 
doubtless  differs  much,  but  it  is  instructive  to  note  in  connection  with 
the  theory  of  the  nerve-impulse  just  suggested,  that  the  wave-motion 
of  light  seems  first  to  induce  recognisable  chemical  changes  in  material 
of  unknown  nature. 

•  This  view  has  been  developed  at  length  in  the  writer's  '  Dynamics  of  Life,' 
London,  Churchill,  1894,  but  was  clearly  stated  by  Herbert  Spencer,  'Elem, 
Biology,'  vol.  i,  1864. 


e2 


DISEASES   OF  THE   NERVES. 


GENERAL  PATHOLOGY. 

Steitctttei!. — Each  individual  fibre  consists  of  a  central  core,  or 
"axis-cylinder,"  surrounded  by  the  "medullary  sheath,"  or  "whibe 
substance  of  Schwann,"  and  a  delicate  membranous 
sheath  enclosing  it.  The  axis-cylinder  is  the  func- 
tional element,  that  which  conducts  the  nerve  im- 
pulses. It  was  long  thought  to  be  homogeneous, 
conducting  as  a  whole,  like  an  insulated  wire,  but 
it  is  now  recognised  to  be  a  compound  structure,  in 
consequence  of  the  recent  researches  of  which  an 
account  has  j  ust  been  given.  The  separate  "  primitive 
fibrils  "  of  which  it  consists  are  sufficiently  numerous 
to  be  of  great  physiological  importance,  an  axon 
according  to  its  size,  containing  apparently  from 
thirty  to  fifty  (see  p.  55).  They  were,  as  has  been 
stated,  described  thirty  years  ago  by  Max  Schultze ; 
but,  although  his  observations  were  not  luiconfimied, 
those  investigators  whose  work  had  most  influence, 
notably  Eanvier,  failed  to  recognise  them.  The  axon 
has  been  practically  regarded  as  simple  until  the  last 
few  years.  Indeed,  the  methods  of  histological  inves- 
of  the  structure  tigation  by  which  its  fibrillary  structure  can  be 
of  nerve-fibres.  I  recognised  are  stiU  outside  the  range  of  ordinary 
later!  Ill  non-  iiiicroscopical  pathology.  For  this,  that  which  was 
mednllated  fibre;  before  discerned  retains  its  importance,  because  it 

S,    sheath  ;      M,  -g  g^^jj  all  that  can  be  applied  to  morbid  changes, 
invehn,  or    white  -"^^  ° 

The  "medullary  sheath  "  (M,  Fig.  32)  consists  of 

myelin,  a  liquid  fatty  material,  supported  by  a  fine 

network  of  homy  substance,  "neurokeratin."     This 

IS,    incision     of  sheath  is  absent  ia  the  "  non-meduUated  "  fibres  of 

Schmidt.     (From  ^]jq  sympathetic.     A  delicate  membrane  surrounds 

a   preparation    of    ,,  ,  .,  ,     ,  ,,  •     •,.  -,       .■,    ,, 

the  nerve-fibre  of  "the  white  sabstance,  the  "  primitive  sheath,  or 
afrog,«tainedwith  "  neurilemma,"  or  "  sheath  of  Schwann  "  (S).  Nuclei 
osmic  aci  .;  ^^^  y[q  at  intervals  within  the  sheath,  between  it  and 

the  myelin.  The  white  substance  is  interrupted  at  regular  distances 
by  what  are  termed  "  nodes," — sometimes,  from  their  discoverer, 
"nodes  of  Tlanvier "  (N).  The  end  of  each  portion,  or  "inter- 
node,"  is  enclosed  by  the  sheath,  through  which  the  axis-cylinder 
passes.     Between   the   incurved  extremities  of  the  sheath  is  a  little 


Fig.  32. — Diagram 


Bubstaace  of 
Schwann;  Ac.axis- 
cyliiider;  n,  nn- 
clens ;  N,  node ; 
incision 


PHYSIOLOGY   OF   NEEVES.  ^'S 

dear  cementing  substance,  shown  by  tbe  fine  dotting  in  tbe  figure. 
Tbere  is  one  nucleus  (n)  to  each  internode,  about  its  middle ; 
hence  they  are  sometimes  called  "internodal  nuclei."  Around 
the  nucleus  is  a  little  protoplasm,  and  it  is  probable  that  a  very 
thin  layer  of  protoplasm  everywhere  lies  between  the  sheath  and 
the  myelin  connecting  that  of  the  adjacent  nuclei,  and  these  with  the 
protoplasm  of  the  nerve-cells  (see  p.  59).  The  internodes  are  shorter 
towards  the  termination  of  a  nerve.  It  is  important  not  to  confound 
these  divisions  with  other  imperfect  indentations  (IS,  Fig.  32,  I), 
often  called  the.  "incisions  of  Schmidt,"  or  "indentations  of 
Lautermann."  *  These  are  oblique,  incomplete  divisions  of  the  white 
substance.  Many  exist  in  each  node.  They  are  believed  by  some 
histologists  to  be  of  artificial  origin,  but  their  uniformity  is  in  favour 
of  their  dependence  on  some  structural  feature. 

The  myelin,  Ranvier  suggests,  must  protect  the  axis-cylinder,  since 
its  almost  hquid  consistence  will  diffuse  pressure  on  the  nerve.  Other 
possible  uses  it  subserves  may  be  suggested,  but  we  have  no  proof  of 
them.  The  nodal  segmentation  evidently  permits  nutrient  material 
to  reach  the  axis-cylinder. 

The  "grey  fibres,"  or  " non-medullated  fibres,"  consist  of  an  axis- 
cylinder,  sheath,  and  nuclei  (Fig.  32,  III),  but  contain  no  myelin. 
They  constitute  the  sympathetic  nerves,  but  some  (also  probably  sympa- 
thetic fibres)  are  found  in  all  the  spinal  nerves.  They  are  absent  from 
the  nerves  of  special  sense  except  the  olfactory,  which  contains  no 
other  fibres. 

The  nerve- fibres  are  tmited  into  "  fasciculi  "  by  delicate  nucleated 
connective  tissue,  and  these  fasciculi  are  similarly  connected  into  larger 
bundles,  while  the  whole  nerve  is  surrounded  by  a  dense  connective- 
tissue  sheath  or  "  perineurium."  Under  this  sheath,  and  extending 
between  its  lamellae  and  amongst  the  individual  nerve-fibres,  there  is  a 
lymph-space  lined  by  flattened  endothelial  cells,  which  probably  plays 
an  important  part  in  the  propagation  of  inflammation.  From  the 
perineurium  a  very  delicate  "sheath  of  Henle"  extends  on  to  the 
ultimate  divisions  of  the  nerve.  AU  these  tracts  of  connective  tissue 
and  spaces  are  continuous,  and  they  convey  the  blood-vessels.  They 
also  contain  nerve-fibres,  "  nervi  nervorum,"  which  are  derived  from 
the  nerve  itself.f  The  sheath  and  connective  tissue  of  each  nerve  are 
thus  part  of  the  area  of  distribution  of  its  own  fibres. 

PHYSiOLoaT  OF  Neeves. — The  first  great  fact  to  be  kept  in  view  is 
that  the  axis  of  each  nerve-fibre  is  the  prolonged  process  of  a  nerve-ceU, 
the  wall  of  the  cell  and  process  being  continued  as  the  sheath  of  the 
nerve-fibre,  the  protoplasm  of  the  cell  becoming  less  and  less  as  the 
process  narrows,  and  the  white  substance,  the  medullary  sheath,  making 

*  They  were  first  described  by  Schmidt  of  New  Orleans,  and  afterwards  by 
Lautei'innnn. 

t  Hoisley,  Roy.  Med.  and  Chir.  Soc,  January  22nd,  1885. 


64  PHYSIOLOGY   OF  NERVES. 

its  appearance  wlieii  the  narrowing  has  reduced  the  process  almost  to 
the  width  of  the  fibre.  This  medullary  sheath  begins  in  the  layer  of 
protoplasm  which  surrounds  the  axis  within  the  neurilemma,  and  which 
can  be  traced,  lessening  as  the  myelin  increases,  for  some  distance. 
Probably  it  does  not  entirely  cease  on  either  side  of  the  myelin 
(see  p.  59).  Thus,  in  the  meduUated  fibres  there  is  a  continuity 
with  the  ceU  of  the  axis,  the  neurilemma,  and  probably  the  protoplasm, 
and  this  continuity  proceeds  from  the  fact  that  the  fibre  is  by 
development  a  part  of  the  cell ;  while  it  is  moreover  found  to  be  so 
vitally,  throughout  life.  It  shares  all  modifications  in  the  nutrition 
of  the  cell,  never  preserving  a  perfectly  normal  state  if  the  nutrition 
of  the  cell  is  changed.  Tet  the  relation  is  less  simple  than  it  seemed 
when  the  axis  was  believed  to  be  but  an  attenuated  prolongation  of 
the  substance  of  the  ceU.  It  has  been  pointed  out  in  the  last  chapter 
that  the  fibrils  which  constitute  the  axis  pass  through  the  cell  and, 
although  they  must  be  considered  part  of  the  cell,  we  are  led  to  look 
rather  to  the  protoplasm,  prolonged  along  the  fibre,  for  the  element  of 
vital  integrity — a  conclusion  in  harmony  with  much  elsewhere. 

The  function  of  nerve-fibreS  is  seen  in  a  different  aspect  under  the 
light  of  the  new  investigation.  As  already  explained,  it  was  formerly 
thought  that  the  cells  produce  and  the  fibres  conduct  what  is  termed 
**  nerve-energy,"  although  fibres  also  can  generate  this  when  they  are 
stimulated,  and  cells  conduct  the  impulse  that  passes  through  them.* 

The  fact  is  now  seen  to  be  that  the  nerve-fibrils  simply  conduct  when 
they  pass  through  the  cell-body,  as  they  do  elsewhere ;  and  it  is  con- 
spicuous when,  for  instance,  a  bundle  of  fibrils  simply  skirts  the  edge  of 
the  cell-body  at  one  corner  in  passing  from  one  process  to  another,  as 
may  occasionally  be  observed. 

That  which  is  true  of  one  fibril  must  be  true  of  all.  The  relations 
are  too  fundamental  for  the  conception  of  differences.  If  any  fibrila 
began  or  ended  in  the  cell- substance,  all  would  do  so.  If  it  is  true  of 
some,  it  must  be  true  of  all,  that  the  function  of  the  cell  is  vital 
nutrition.  Where  the  fibrils  begin  as  such,  as  conducting  structures 
with  the  constitution  susceptible  to  the  same  changes  throughout, 
whether  this  "  beginning  "  is  a  change  of  constitution  or  of  structure 
— ^there  we  must  look  for  the  source  of  the  energy  that  is  conducted. 
But  we  have  not  yet  learned  how  to  look. 

The  division  of  nerve-fibres  where  they  terminate,  as  in  muscle,  or 
begin,  as  in  the  skin,  must  be  regarded  as  separation  or  union  of  axis- 

*  The  double  similarity  of  the  function  of  the  cells  and  the  fibres  was  further 
insisted  on  in  the  last  edition  of  this  book  in  words  that  are  worth  quoting,  because 
they  show  how  significant  are  the  facts  of  function  as  well  as  of  structure:  — 

*'The  process  of  the  cell  it-^elF  must  conduct,  and  the  cell  must  itself  conduct. 
Each  motor  cell  of  the  cord  is  part  of  the  path  through  which  the  nerve-energy 
from  the  brnin  passes.  That  which  passes  through  must  be  conducted,  however  it 
may  be  chanrred  in  amount.  Even  if  it  is  renewed,  we  cannot  so  distinguish  thia 
function  of  the  cell  from  that  of  the  fibre  as  to  say  that  there  is  not  conduction." 


LE8I0NS    OF   NERVES.  65 

fibrils  or  of  groups  of  them.  This  has  been  already  erplainecl,  and 
other  points  have  been  mentioned  in  the  last  chapter  which  concern  the 
action  of  the  nerves,  in  so  far  as  this  can  be  discerned  with  distinctness. 

Lesions  of  Nerves. — Seconda/ry  Degeneration. — A  nerve-fibre 
■undergoes  destructive  changes  whenever  it  is  separated  from  the 
cell  from  which  it  springs,  i.  e.  the  body  of  the  cell  of  which  it  is 
vitally  a  part.  Formerly,  importance  was  attached  to  the  fact  that  as 
a  rule  the  degeneration  is  in  the  direction  of  conduction,  i.  e.  from  the 
cell  from  which  the  nerve-fibre  conducts  nerve-impulses.  This  must 
be  so  because  each  fibre  belongs  only  to  one  cell.  The  fact  is,  however, 
conspicuous,  because  it  is  only  in  the  long  axis-processes  that  the 
direction  of  degeneration  can  be  observed.  The  short  dendrons 
degenerate  from,  though  they  usually  conduct  towards,  the  cell-body  ; 
and  so  also  do  the  long  sensory  fibres,  axis-processes  of  the  cells  of  the 
spinal  ganglia.  These  were  formerly  regarded  as  inexplicable  excep- 
tions to  the  correspondence,  when  the  impulses  were  thought  to  be 
produced  in  the  ceU.  We  can  now  see  that  they  are  instances  of  the 
general  condition. 

The  degeneration  is  commonly  termed  "secondary,"  because  it  is 
dependent  on  a  "primary"  lesion  of  another  kind — as  division  or 
desti-uction  of  the  cell-body  of  the  nerve.  Degeneration  also  follows 
many  slighter  lesions  such  as  nerve  compression,  transient  or  exten- 
sive, or  local  inflammation  and  the  like  ;  and  this  may  differ  only 
in  degree  from  that  which  follows  division.  It  must  be  more  than 
a  difference  of  degree,  however,  when  there  is  no  recovery.  The 
secondary  degeneration  is  often  called  "  Wallerian,"  from  the  name  of 
the  pathologist  who  first  studied  it.  It  is  of  great  importance  practi- 
cally and  theoretically.  The  medullary  sheath  breaks  up  into  segments, 
and  these  into  smaller  and  smaller  fragments,  and  the  minute  globules 
and  granules  are  ultimately  removed  from  the  nerve- sheath,  and 
when  the  degeneration  is  complete  the  axis-cylinder  also  perishes. 
The  process  is  not  one  of  mere  decay,  but  is  an  active  one  in  which  the 
nuclei  of  the  fibre,  and  their  protoplasm,  assume  an  exuberant  energy  of 
growth,  while  the  protecting  myelin  sheath  and  the  essential  conducting 
axis  perish,  apparently  at  the  same  time.  In  connection  with  this  it 
may  be  remembered  that  in  the  central  organs,  the  connective  tissue 
overgrows  as  sclerosis  when  the  fibres  decay.  Developmental  relations 
justify  us  in  connecting  the  two  sets  of  facts.  In  all  organs,  indeed, 
we  can  perceive  the  fact,  though  most  clearly  in  the  nerve- structures, 
that  "parenchymatous  "  decay  and  adventitial  growth  go  hand  in  hand. 

In  nerve-fibres  the  aspect  of  the  process  of  degeneration  can  be 
better  perceived  than  in  any  other  structn.es,  and  it  has  been  carefully 
etudied  in  animals,  chiefly  by  Eanvier.  The  most  important  facts  are 
illustrated  in  Fig.  33,  in  which  the  examples  have  been  selected  from 
Ranvier's  figures  and  reduced  to  one  tint.  First  of  all  the  nuclei 
are  increased  in  size  (A  n,  B  w)  ;  the  amount  of  protoplasm  about 
VOL.    1.  5 


66 


LESIONS  OP   NERVES. 


them  is  greater  than  normal,  and  is  granular;    there  is  in  places 
a  local  increase  in  the  amount  of  protoplasm  within  the  sheath,  com- 


Fia.  33. 


Fig.  34. 


Ifi 


Pig.  33. — A-J,  degeneration  of  nerve-fibres  (osraic  acid  and  carmine 
staining).  A,  from  sciatic  of  rabbit  four  days  ufter  section ;  B,  C.  same, 
fifty  hours  atter  section ;  D,  a  fibre  stained  with  carmine  only,  to  show  the 
axis- cylinder;  E,  F,  G,  fibres  from  pigeon  three  days  after  section;  H,  two 
fibres  from  pneumogastric  of  rabbit  six  days  after  section;  J,  a  lymphatic 
cell  from  interstitial  connective  tissue,  containing  globules  of  myelin  which 
it  has  taken  up.  In  all  the  figures— n,  n,  nuclei ;  x,  x,  constrictions  of  the 
mvolin  produced  by  the  growth  of  the  protoplasm  ;  ao,  axis- cylinder, 

"K,  L,  regeneration  of  nerve-fibres.  K,  from  pneumogastric  of  rabbit 
se^  eiitytwo  days  after  section  ;  L,  from  sciatic  of  rabbit  ninety  days  after 
section;  e,  rounded  end  of  white  substance  of  central  end  of  nerve;  #, 
sheath;  na,  new  axis-cylinder.  In  L  are  two  globules  of  myelin  remaining 
from  the  degeneration  of  the  old  fibre. 

Fig.  34. — Degenerating  fibres  from  cutaneous  nerves  of  man.     (After 
Pifres  and  Vailltnd.)     A,  from  near  a  bedsore  in  a  case  of  fracture  of  the 
skull ;  B,  C,  D,  from  the  fifth  nerve  in  a  case  of  neuralgia  and  ulceration  of 
the  lip;  n,  n,  nuclei.     In  A  the  protoplasm  and  nuclei  are  increased,  and 
the  myelin  is  breaking  up,  the  processes  liaving  ])roceeded  furthest  in  the 
neighbourhood  of  the  nucleus  in  the  middle  of  the  fibre ;  in  B  the  segmenta- 
tion has  gone  on  to  the  formation  of  globules,  which  in  C  are,  for  tlie  most 
part,  small,  and  many  have  been    removed,  so  that  the  fibre  is  narrow; 
while  in  D  all  the  products  of  degeneration  have  been  removed  from  con- 
siderable tracts  of  the  sheath, 
pressing  the  myelin  (A  x,B  x,G  x).   The  nuclei  then  become  detached 
from  the  sheath  ;  the  protoplasm  everywhere  increases,  and  encroaches 
on  the  myeUn,  until  here  and  there  it  meets  across  the  tube,  com- 


GLNERAL    PATHOLOGY.  67 

pletely  separating  tlie  myelin  (A,  lower  part ;  B,  upper  part),  and  "with 
the  myelin  the  axis-cylinder  (D).  This  process  then  goes  on  with 
increased  rapidity ;  the  myelin  is  broken  up  into  smaller  and  smaller 
fragments  (C,  E,  P),  which  become  globular  in  the  watery  protoplasm, 
just  as  oil  forms  globules  in  water.  The  nuclei  meanwhile  continue  to 
enlarge,  and  then  divide,  first  the  nucleolus  and  then  the  whole  nucleus 
(F).  The  two  nuclei  may  again  divide,  until  (as  in  Gr)  there  are  four 
or  more  nuclei  ia  each  intemode,  instead  of  one  only  as  in  health. 
The  small  globules  of  fatty  myelin  seem  to  become  changed  in  chemical 
composition,  since  they  are  stained  less  deeply  by  osmic  acid.  Ranvier 
suggests  that  their  fatty  matter  may  undergo  a  process  of  saponification. 
Ultimately  they  seem  to  pass  through  the  sheath,  are  taken  up  by  con- 
nective-tissue cells  and  lymphatic  cells  in  the  vicinity  (as  in  J),  and  are 
gradually,  for  the  most  part,  removed.  By  the  time  the  myelin  is  in 
small  globules  the  nuclei  cease  to  multiply.  On  the  removal  of  the 
products  of  degeneration  the  sheath  shrinks,  and  looks  empty  in 
places ;  but  here  and  there  it  is  enlarged  by  the  nuclei,  protoplasm, 
and  a  few  remaining  myelin  globules  (H).  Hence  in  transverse  section 
many  small  sheaths  are  seen,  with  a  few  of  larger  size  where  they  have 
been  cut  across  at  these  swellings. 

In  the  rabbit  the  first  changes  are  visible  at  the  end  of  twenty-four 
hours  ;  the  first  complete  interruption  of  the  myelin  and  axis-cylinder 
occurs  about  the  end  of  the  second  day ;  the  process  of  destruction  is 
considerably  advanced  at  the  end  of  the  fourth  day,  and  is  finished, 
and  the  multiplication  of  the  nuclei  ceases,  towards  the  end  of  the 
second  week.  In  peripheral  nerves  the  changes  seem  to  progress 
from  the  lesion  to  the  periphery,  but  within  the  central  nervous 
system  they  are  said  to  begin  at  the  same  time  in  all  parts  of  the 
fibre.  It  is  uncertain  to  what  extent  this  difference  is  real  or  is  only 
apparent,  but  the  process  goes  on  most  rapidly  at  the  periphery. 

At  the  end  of  the  second  day,  in  the  rabbit,  stimulation  of  the 
nerve  by  electricity  below  the  lesion  no  longer  causes  muscular  con- 
traction, the  disappearance  of  electrical  excitability  coinciding  with  the 
first  complete  segmentation  of  the  myelin  and  axis-cylinder. 

A  curious  fact,  imfortunately  more  interesting  in  pathology  than  in 
practice,  is  that  the  degeneration  of  a  divided  nerve  does  not  occur  if 
the  ends  are  brought  quickly  into  perfect  apposition.  It  is  so  in 
animals,  and  probably  also  in  man.*  Immediate  replacement  may 
occur  in  some  incised  wounds,  and  thus  the  fact  is  explained  that 
simple  division  is  a  less  serious  injury  than  a  grave  contusion. 

The  axis-cylinder  seems  thus  to  undergo  these  changes  as  a  whole, 
although  we  know  that  it  is  a  group  of  fibrils.  But  we  have  seen  that 
the  conducting  substance  of  these  fibrils,  and  that  which  separates  it 
(and  may  be  conceived  as  analogous  to  the  myelin  around  the  whole 
axis-cylinder),  differ  but  slightly  in  consistence.   Both  are  nearly  fluid, 

•  See  Bowlby, '  Injuries  and  Diseases  of  Nerves,'  p.  32. 


68  LESIONS    OP   NERVES. 

and  so  break  up  into  globules,  although  they  differ  in  constitution  in  a 
manner  and  degree  that  subserve  entire  difference  in  function,  and 
permit  reagents  to  produce  artificial  differences  in  aspect  and  cha- 
racters misleading  if  simply  transferred  to  the  living  state,  and  yet  the 
revelation  of  an  essential  difference  of  absolute  importance. 

The  process  is  thus  the  result  of  an  active  growth  of  the  nuclei  and 
protoplasm  of  the  nerve,  i.  e.  of  the  cellular  elements  of  which  the 
nei-ve  is  composed.  Why  does  this  occur  ?  The  determining  cause  is 
the  interruption  of  the  continuity  with  the  cell-body  or  the  destruction 
of  this.  Ranvier  connects  the  destructive  growth  of  the  protoplasm 
with  the  loss  of  function  in  the  axis,  and  suggests  that  normally  the 
activity  of  this  restrains  the  vital  energy  of  the  cell-elements. 

These  changes  are  often  attended  with  others  outside  the  fibres, 
such  as  we  regard  as  evidence  of  inflammation.  Several  observers 
have  describedv  increase  of  nuclei  and  accumulation  of  leucocytes  in  the 
interstitial  connective  tissue,  and  even  in  the  nerve-sheath,  with 
dilatation  of  the  blood-vessels.  Such  changes  are,  indeed,  intense  at 
the  primary  lesion ;  their  degree  in  the  nerve  below  the  lesion  varies 
and  seems  to  be  proportioned  to  that  in  the  primary  process.  This  is 
another  mode  of  statiuL,'  the  important  fact  that  the  irritative  character 
of  the  secondary  process  is  determined  by  the  irritative  nature  of  the 
primary  disease.  We  may  say,  if  we  like,  that  in  the  slighter  degrees 
it  is  distinctly  inflammatory  in  aspect  in  proportion  to  the  signs  of 
inflammation  at  the  primary  lesion.  But  this  is  only  another  mode  of 
stating  one  part  of  the  general  fact,  and  the  importance  of  the  transmis- 
sion of  the  character  of  the  process  cannot  be  too  strongly  insisted  on. 

The  process  of  secondary  degeneration  occixrs  more  slowly  in  the 
rabbit  than  in  a  bird,  and  seems  to  be  still  slower  ia  man,  in  whom  it 
is  probable  that  complete  segmentation  does  not  occur  until  between 
the  fourth  and  eighth  days.  It  is  certain  than  an  identical  process 
occurs  in  man.  Changes  in  peripheral  nerves  near  bedsores  and  in 
the  fifth  nerve,  found  by  Pitres  and  Vaillard,  are  shown  in  Fig.  34,  a 
comparison  of  which  with  Fig.  33  will  show  the  identity  of  the  process. 
It  is  highly  probable  that  after  complete  division  of  a  nerve  in  man  the 
changes  are  the  same  as  in  animals.  But  the  most  common  lesion  is, 
or  involves,  inflammation,  and  the  process  of  degeneration  is  thus 
greatly  modified  by  the  character  of  the  primary  lesion,  and  in  time  as 
well  as  in  character.  In  the  latter  cases,  which  are  chiefly  those  of  focal 
neuritis,  the  process  cannot  be  the  same  as  that  which  follows  sec- 
tion of  a  nerve.  The  change  in  irritability,  which  will  be  more  fully 
described  in  the  account  of  the  symptoms,  is  a  slow  depression,  some- 
times moderate  in  degree.  The  depression  may  be  preceded  by  an 
increase  in  irritability.  In  such  cases  there  can  be  no  complete  seg- 
mentation of  the  nerve-fibres.  There  must  be  a  gradual  alteration  in 
the  molecular  nutrition  of  the  axis-cylinder,  changing  its  excitability. 
Even  in  severe  cases  there  is  not  usually  a  sudden  loss  of  irrita- 
bility ;  the  current  necessary  for  stimidation  has  to  be  made  stronger, 


GENERAL   PATHOLOGY.  Q'J 

until  at  last  in  seven  or  eight  days  the  strongest  endurable  current  fails 
to  cause  muscular  contraction.  But  we  cannot  infer,  from  this  alone, 
that  there  is  an  actual  interruption  of  the  axis-cylinder.  A  stronger 
current  might  still  excite  the  nerve,  because,  as  we  shall  presently  see, 
when  a  nerve  is  being  regenerated,  an  axis-cylinder  may  conduct,  and 
still  not  be  excitable  by  currents  of  ordinary  strength. 

All  severe  changes  in  the  nutrition  of  the  fibres  involve  the  intra- 
muscular nerve-endings  in  the  same  degree.*  The  evidence  of  this  is 
that  the  faradic  irritability  of  the  muscles  (which  depends  on  the 
nerves  within  them)  presents  changes  quite  similar  to  those  of  the 
nerve-trunk.  But  this  is  not  always  true  in  slight  changes  of  nutrition 
of  the  nerve.  We  shall  presently  see  that  the  slight  alterations  of  irri- 
tability in  the  nerve  and  muscle  do  not  always  correspond.  The  nerves 
terminate  in  structures  of  special  nature,  and  these  may  well  have  some 
shght  degree  of  nutritional  independence. 

These  secondary  destructive  changes  occur  in  the  peripheral  part  of 
the  divided  nerve.  But  the  part  that  remains  continuous  with  the  cell 
does  not  always  preserve  a  normal  state.  Changes  occur  in  it,  espe- 
cially near  the  lesion,  which  are  still  the  subject  of  study,  and  of  which 
more  must  be  known  before  their  significance  can  be  determined.  In 
a  divided  nerve  the  changes,  which  are  mainly  those  of  ordinary, 
often  incomplete,  degeneration  in  isolated  axis-cylinders,  affect  the 
centripetal  (sensory)  fibres  far  more  extensively  than  the  centrifugal. 
Later  on  the  cells  connected  with  both  suffer,  the  nucleus  shrinking 
and  the  protoplasm  to  some  extent  becoming  disintegrated.f 

Regeneration  may  occur  in  the  nerve  after  the  degeneration  is  over. 
It  is  a  slow  process,  occupying  the  second,  third,  and  fourth  month 
after  division.  According  to  Eanvier,  and  as  Waller  thought,  it  occurs 
always  by  the  growth  of  new  axis-cylinders  from  the  central  end  of  the 
nerve  (see  Fig.  33,  K  and  L),  which  ultimately  become  covered  with 
myelin.  One  or  more  new  fibres  may  spring  from  each  central  fibre, 
and  these  may  subdivide.  All  are  enclosed  in  a  sheath  which  is  con- 
tinuous with  that  of  the  central  end  (Fig.  33,  K,  «.) .  We  must  assume 
that  only  some  of  these  ax  is- cylinders  persist  and  achieve  functional 
permanence.  But  the  whole  of  this  subject  needs  re-investigation  in 
the  light  of  the  multiple  constitution  of  the  axis-cylinder.  J  Some- 
times these  fibres  twist  about,  and  even  turn  back  and  grow  upwards, 
probably  in  the  direction  of  least  resistance.     In  animals  new  fibres 

•  Fully  described  and  figured  by  Babes  and  Mariiiesco,  'Babes'  A  tins  Path. 
Ilistol.  des  Nervensystems,'  part  5. 

t  SeeHomen, '  Ziegler's  Beitrage,'  viii,  and  'Babes' Atlas,'  part  2.  R.  A.  Fleming 
('  Brit.  Med.  Journ.,'  1896,  ii,  p.  918)  lays  great  stress  on  the  degeneration  of  the 
fine  (vaso-motor  ?)  fibres  above  the  lesion,  and  describes  also  a  considerable  con- 
nective-tissue overgrowth  in  the  same  part. 

X  The  precise  renewal  of  these  fibrillary  conducting  paths  cannot  be  ascertained 
in  animals,  and  in  man  the  amount  of  damage  that  is  renewed  is  far  smaller.  Facts 
are  needed  regarding  minute  differential  localisation  after  recovery  from  an  injury, 
compared  with  the  normal. 


70  LESIONS    OP   NERVES. 

may  grow  throtigli  a  consideraLle  extent  of  cicatricial  tissue  between 
the  divided  ends  of  a  nerve,  but  in  man  it  is  doubtful  wbetlier  regene- 
ration of  a  divided  nerve  occurs  unless  tbe  extremities  are  brought  in 
contact,  or  at  least  close  proximity.  Some  investigators  believe  that 
there  is  a  formation  of  fibres  in  the  peripheral  extremity  independ- 
ently of  the  growth  of  new  fibres  from  the  central  end.*  In  cases  of 
slight  injury,  regeneration  occurs  more  readily  ;  in  these  it  is  probable, 
as  we  have  just  seen,  that  degeneration  has  been  incomplete.  The 
regenerated  nerve- fibres  regain  some  conducting  power  while  they  are 
still  much  narrower  than  normal,  and  before  they  can  be  excited  by 
electricity.f 

Muscles. — The  degeneration  of  the  motor  nerves  is  attended  by 
changes  in  the  nutrition  of  the  muscles.  These  commence  in  or  after 
the  second  week.  The  muscular  fibres  become  narrower,  and  may  be 
reduced,  ultimately,  to  one  third  of  their  former  width.  An  increase  in 
width  has  been  said  to  precede  the  narrowing  for  a  few  days. J  The 
transverse  striation  becomes  less  distinct,  and  the  striae  seem  to  be  nearer 
together  than  in  health.  The  fibres  may  become  cloudy  or  granular, 
but  do  not  present  actual  fatty  degeneration  except  in  some  very  acute 
cases.  If  no  regeneration  of  the  nerve  takes  place,  the  transverse 
striation  gradually  disappears,  and  may  be  replaced  by  a  longitudinal 
striation,  or  the  fibres  may  undergo  certain  chemical  changes,  and  pre- 
sent a  peculiar  glassy  appearance,  which  has  been  called  "vitreous 

*  a.  g.  Neumann,  Mayer,  &c.  A  full  abstract  of  their  observations  is  given  by 
Bowiby  (loc.  cit.),  and  also  by  Allen  Starr  in  the  Middletpn  Goldsmith  Lectures 
for  1887  ('  New  Yoi  k  Med.  Record,'  Febrdary,  1887).  By  some  the  new  axis- 
cylinders  are  said  to  be  formed  by  elongation  of  the  fragments  of  the  old  axis ;  by 
others  from  the  nuclei  of  the  sheath  of  Schwann,  but  these  have  been  found  by 
others  to  take  no  part  in  regeneration  (von  Notthaft,  see  below).  But  Ranvier'a 
careful  investigations  have  been  fully  confirmed  by  the  minute  researches  of  Vanlair 
(*  Arch,  de  Biologic,'  1885),  and  by  the  more  recent  experiments  of  Stroelie 
.(*  Ziegler's  Beitrage,*  1893),  and  it  is  difficult  to  understand  that  perfect  axis- 
cyliu'iers  should  be  formed  and  remain  unexcitable,  as  the  peripheral  segmeut 
certainly  does.  Structures  may  be  formed  resembling  axis-cylinders  that  are  nob 
really  capable  of  the  proper  function.  The  weight  of  Ranvier's  undisproved  and 
confirmed  observations  is  very  great.  His  conclusion  that  there  is  no  discontinuous 
formation  of  fibres  has  been  also  confirmed  by  von  Nottliaft  (' Zeitsch.  f.  Zool.,' 
1892,  Bd.  Iv).  It  is  possible  that  the  process,  in  the  peripheral  part  of  a  nerve,  is 
influenced  by  the  connection  with  other  nerves  and  the  recurrent  influence  of 
anastomosing  fibres. 

t  Ziegler,  in  a  recent  paper  ('Arch.  f.  klin.  Chirurg.,'  1896),  reasserts  the  ahan- 
doned  view  that  the  old  axis-cylinder  plays  no  part  in  regeneration,  the  active  agent 
in  whli-,h  is  a  nucleated  protoplasm  which  he  states  to  be  an  outgrowth  of  the 
sheatl:  of  Scliwann  (this  probably  refers  to  the  protoplasm  mentioned  on  p.  51). 
F'l-oiji  this  he  describes  the  development  of  a  primary  protoplasmic  fibre,  from 
wliich  are  differentiated  not  only  a  new  axis-cylinder  but  also  a  new  sheath  of 
Schwann  and  medullary  substance.  This  new  axis-cylinder  is  ultimately  joined  to 
the  old  stump.  These  statements  have  not  as  yet  received  confirmation  by  other 
observers. 

J  Steinert, '  Verb.  Phys.  Ges.  Wurzburg,'  1888,  No.  10. 


SYMPTOMS. 


71 


U  generation."  During  the  progress  of  the  changes  in  the  fibres,  the 
nuclei  of  the  sarcolemma  and  of  the  interstitial  tissue  are  increased  in 
number,  and  develop  into  fibrous  tissue,  so  that  ultimately  the  muscular 
fibres  are  separated  by  considerable  tracts  of  connective  tissue,  and  a 
state  of  cirrhosis  results.  If  regeneration  of  the  nerve  occurs  the 
muscular  changes  are  arrested,  and  the  normal  condition  is  slowly  re- 
stored. When  recovery  of  the  nerve  is  slow,  and  the  secondary  process 
in  the  muscle  considerable,  the  fibres  remain  small,  the  amount  of  con- 
nective tissue  is  permanently  increased,  but  undergoes  contraction,  so 
that  the  muscle  is  for  a  long  time  smaller  than  normal,  and  its  natural 
bullc  may  never  be  regained.  If  no  regeneration  of  the  nerve  occurs, 
the  muscular  fibres  gradually  disappear ;  fibrous  tissue  takes  their 
place,  and,  slowly  contracting,  permanent  shortening  may  result. 
Similar  shortening  sometimes  occurs  when  there  is  partial  recovery  of 
the  nerve  and  muscle.  In  most  lesions  of  nerves,  other  than  actual 
division,  some  fibres  recover,  even  though  others  are  permanently 
destroyed.  The  muscular  degeneration  is  the  result  of  that  of  the 
motor  nerves,  as  described  at  pp.  66,  67. 


Symptoms  of  Neeve  Injury  and  Degeneration. — The  symptoms 
that  attend  the  lesions  of  motor  nerves  and  the  consequent  degene- 
ration are  of  great  importance.  The  lesion  of  the  nerve  causes  para- 
lysis of  the  muscles  supplied  by  it,  due  to,  and  in  proportion  to, 
the  interference  with   the   conductiag    power   of    the   nerve.      The 


WEEKS   I 


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N 


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\\ 


' iiiiiil IIIIMIIIIIIIIIIKIIIIIIIIIIMIIIIIKJ 


Pig.  35. — Type  of  degenerative  reaction  in  a  case  of  nerve-ininry  of  inode- 
rate  severity.  (After  Erb.)  Muscle  irritability  lowered  from  middle  of 
first  wreek ;  faradic,  extinct  in  middle  oF  third,  reappearing  in  ninth; 
voltaic,  increased  from  middle  of  second  week  until  tenth,  then  de- 
pressed until  t'onrteenth.  Nerve  irritability  (changed  alike  to  both 
currents)  is  lowered  from  middle  of  first  week,  lost  Ht  end  of  second, 
reappearing  at  seventh.     Power  lost  until  end  of  fifth  week. 

tnuscles  at  once  become  flabby  from  loss  of  tone,  and  to  this  atony 
actual  wasting  is  added  in  the  course  of  a  few  weeks.  The  wasting 
is  due  to  the  reduction  in  size  of  the  muscular  fibres.  If  the  sensory 
nerve-fibres  are  not  interrupted,  the  muscles  become  tender  to  the 
touch,  and  pain  is  caused  by  their  strong  contraction,  due  probably  to 
the  effect  of  interstitial  inflammation  on  the  sensory  nerves,  which  end 
in  the  connective  tissue. 

The  most  important  symptoms  are  those  that  are  afforded  by  elec- 


72  LESIONS    OF    SERVES. 

trical  examination  of  the  muscles  and  nerves,  since  they  enable  the 
degeneratiye  changes  to  be  ascertained  and  followed  during  life.*  The 
alterations  in  the  electrical  reaction,  consequent  on  this  degeneration, 
have  been  already  briefly  mentioned  (p.  29),  but  must  now  be  described 
in  greater  detail.  The  rapid  degeneration  of  a  nerve,  which  follows  a 
severe  lesion,  is  attended  by  a  loss  of  irritability  on  electrical  stimula- 
tion, the  loss  being  the  same  to  faradism  and  voltaism.  After  such 
lesions  as  are  common  in  man — neuritis,  for  instance, — there  is  no 
sudden  loss,  such  as  occurs  after  injury  of  a  nerve  in  an  animal,  when  the 
nerve  becomes  segmented,  but  there  is  a  more  or  less  rapid  diminution 
of  excitability,  and  this  goes  on  until  no  stimulation  can  be  produced, 
even  by  a  strong  current.  The  progressive  changes  in  irritability  may 
be  conveniently  represented  on  a  chart.  Fig.  35  shows  the  typical 
course  of  the  changes  of  irritability  in  a  case  of  moderate  severity.  In 
the  muscle  (M)  a  fall  of  irritability  (due  to  the  degeneration  in  the 
nerve-endings)  occurs  simultaneously  with  that  in  the  nerve-trunk  (N), 
and  the  f  aradic  excitability  becomes  extinct  at  the  same  time  in  both 
nerve  and  muscle.  The  fall  in  voltaic  irritability  is  quickly  arrested 
by  the  change  in  the  muscular  fibres,  through  which  they  soon  become 
more  excitable  than  normal  to  the  voltaic  current.  This  change  usually 
occurs  during  the  second  week,  and  the  irritability  continues  to  increase 
during  the  third  and  fourth  weeks.  At  its  maximum  it  may  amount 
to  three,  four,  or  five  cells  of  the  battery,  i.  e.  contraction  can  be 
obtained  in  the  paralysed  muscles  with  a  current  weaker  by  so  many 
cells  than  is  necessary  to  cause  contraction  in  a  corresponding  un- 
affected part.  The  further  course  of  the  changes  in  irritability  depends 
on  the  severity  of  the  lesion  and  the  intensity  of  the  degeneration.  In 
a  case  of  moderate  degree,  such  as  is  shown  on  the  chart,  nerve  irrita- 
bility reappears  about  the  end  of  the  second  month,  usually  after  some 
voluntary  power  is  regained.  It  is  at  first  low,  so  that  a  strong  current 
is  required.  It  gradually  increases,  but  for  a  long  time  continues  a 
little  below  the  normal  degree.  This  return  of  nerve  irritability  is 
accompanied  by  a  corresponding  return  of  faradic  irritabiUty  in  the 
muscles  (i.e.  in  the  intra-muscular  nerves).  The  increase  of  voltaic 
irritability  often  persists  long  after  recovery  of  power,  but  it  lessens 
as  faradic  irritability  returns,  and,  as  shown  in  the  chart,  it  may  fall 
below  the  normal  before  it  ultimately  regains  its  original  degree. 

Slight  changes  in  irritability  can  be  ascertained  only  by  a  comparison 
with  the  corresponding  part  on  the  other  side  in  the  same  individual. 
Moreover,  when  we  speak  of  excitability  being  "  lost,"  we  mean  that 
we  can  obtain  no  stimulation  by  any  strength  of  current  that  can  be 
borne.  The  sensitiveness  of  the  skin  varies  in  different  j^ersons  and  in 
different  parts.   The  resistance  of  the  skin  also  varies  ;  the  greater  it  is 

*  For  !i  careful  analysis  of  abnormal  electrical  reactions  in  muscle  and  nerve  see 
Douiner,  '  Bulletin  Offic.  de  la  Soc.  fran9.  d'Electrotherapie,*  1897.  The  author  lays 
especial  stress  on  the  importance  of  recording  the  nature  of  the  excitation  and  the 
character  of  ihe  muscle-curve  produced. 


SYMPTOMS. 


73 


the  stronger  must  be  the  current  used  to  secure  the  action  on  the  nerves 
below.  Hence  the  importance  of  ascertaining  what  current  is  actually- 
passing  by  the  use  of  a  galvanometer.  It  may  be  used  once  to  gain  in- 
formation as  to  the  significance  of  the  number  of  cells  that  constitutes 
the  evidence  of  the  difference  between  the  two  sides  when  the  sensitive- 
ness to  the  pain  which  is  caused  prevents  the  use  of  the  instrument  in 
all  measurements. 

If  the  lesion  is  very  severe,  so  that  there  is  no  recovery,  and  no  re- 
generation of  the  nerve,  the  loss  of  nerve  irritability,  and  of  the 
faradic  muscular  irritability,  is  permanent.  The  increase  in  voltaic 
irritability  persists  for  months,  and  then  gradually  falls  as  the 
muscular  fibres  waste,  and  becomes  lower  and  lower  (see  Fig.  36), 


WEEK^r    3         10 


20 


30 


40         50 


60 


70 


eo 


90 


:.-. 

M 

•.F 

'V 

-~-i: 



Icnc: 

r-^wu-i; 

■'-■ 

c 

N 

■•% 

r\oTpuiJk 

line^ 

P 

Pig.  36. — Type  of  reaction  in  a  case  of  complete  and  permanent  damage  to 
a  nerve.  (After  Erb.)  Early  course  as  in  the  last  diagram;  no  return  of 
power,  nerve  irritability,  or  faradic  irritability  in  the  muscle.  The  early 
increase  in  voltaic  irritability  gradually  lessens,  and  at  the  end  of  ten 
months  falls  below  the  normal,  but  is  not  extinct  until  nearly  two  years. 

In  this  and  the  foUowina:  figures  the  normal  degree  of  irritability  (ascer- 
tniiied  from  the  healthy  side)  is  represented  by  the  finely  dotted 
horizontal  line  (w.^.) ;  faradic  irritability,  P,  by  a  line  of  larger  dots; 
voltaic  irritability,  V,  by  a  broken  line;  M,  muscle;  N,  nerve;  P, 
power  of  voluntary  contraction,  its  degree  shown  by  the  vertical  lines; 
the  asterisk  marks  the  occurrence  of  the  lesion.  The  vertical  divisions 
represent  time  intervals.  (Many  of  these  indicating  letters  are  given 
only  in  subsequent  figures.) 

until  ultimately  no  reaction  can  be  obtained,  the  fibres  having  perished. 
It  does  not  become  extinct  until  at  least  a  year  has  elapsed,  and  some- 
times (as  in  the  chart)  only  towards  the  end  of  the  second  year. 
Often,  when  no  contraction  can  be  produced  on  the  first  attempt,  after 
two  or  three  applications,  distinct  contractions  are  obtained. 

The  changes  we  have  hitherto  considered  are  in  the  degree  of  irrita- 
bility, "  quantitative  "  changes.  But  the  quantitative  increase  in  the 
muscular  irritability  is  often  accompanied  by  a  change  in  the  order  of 
response,  according  to  the  pole  that  is  applied  and  the  strength  of 
current — a  "  qualitative"  or  "polar"  change.  We  have  already  con- 
sidered its  general  characters  (p.  31),  and  have  seen  that  it  consists 
in  an  undue  readiness  of  response  at  the  positive  pole  (anode)  com- 
pared with  that  at  the  negative  poll  (kathode)  (Fig.  37,  B  and  C), 
the  muscle  being  normally  the  more  sensitive  to  the  latter  (Fig.  37,  A). 
Writing  CI.  for  the  closure  of  the  circuit,  0.  for  its  opening,  and  C. 
for  contraction,  the  normal  reaction  is — 


74 


LESIONS  OF   NERVES. 


1.  K.CLC. ;  2.  {;^^Q^^-.'  3.  K.0.0. 
In  disease — 

1.  I  f  ^^q^q'  .  2.  An.O.C. ;  3.  K.O.C. :  or  even 
1.  An.Cl.C;  2.  KCl.C. ;  3.  K.O.C;  4.  An.O.C. 

Tliis  qualitative  change  is  at  times  absent,  especially  in  cases  of 
neuritis.  It  may  be  slight,  and  only  amount  to  an  equal  kathodal 
and  anodal  excitability.  It  is  practically  constant  when  a  nerve  has 
been  actually  destroyed.  On  the  other  hand,  it  may  be  present  and 
significant  where  less  acute  disease  has  not  yet  caused  obtrusive 
wasting.  Even  when  there  is  a  marked  quantitative  change,  the 
kathodal  closure  contraction  may  still  occur  first.  When  the  change 
is  present  it  is  only  in  the  muscles,  and  it  must  depend  on  the  muscular 
fibres  themselves.  In  the  motor  nerve  the  kathodal  response  is  always 
the  first,  although  a  qualitative  change  has  been  detected  in  degenerated 
sensory  nerves. 

The  muscular  contractions  which  occur  thus  with  undue  readiness 
differ  from  normal  contractions,  excited  through  the  nerves,  in  their 
distinctly  deliberate  character.     Instead  of  the  quick,  lightning-like 


Ka    An  Ka      An 


Ka      Art 


Ka     An 


Ko.     Ai 


Fig.  37. — Tracings  of  the  muscular  contractions  in  nerve  degeneration.  (After 
Erb.)  Ka,  katliodal  closure  contraction;  An,  anodal  closure  contraction. 
A,  tracing  in  liealth;  Ka  nouch  stronger  than  An;  contractions  sudden.  B, 
tracing  in  nerve-degeneration  with  moderate  current.  An  much  greater 
than  Ka,  the  latter  scarcely  visible.  Contraction  slower,  shown  by  the  more 
sloping  upstroke.  C,  the  same,  with  a  stronger  current.  Ka  greater,  but 
still  less  than  An ;  the  slow  character  of  the  contraction  and  its  long  duration 
well  marked. 

contraction,  the  movement  is  distinctly  longer  in  reaching  its  maximum 
and  longer  in  its  duration.  The  recognition  of  this  feature  is  easy, 
and  often  of  practical  importance  when  there  is  doubt  whether  tlie 


SYMPTOMS. 


75 


voltaic  current  is  acting  through  the  nerve-twigs,  or  on  the  muscular 
fibres  themselves.  Sometimes  a  slight  abnormal  tetanic  contraction 
during  the  passage  of  the  current  also  occurs.  During  the  period  of 
increased  voltaic  irritability  the  mechanical  excitability  of  the  fibres  is 
often  increased.  If  they  are  directly  percussed,  they  respond  with  a 
distinct  slow  contraction  (Erb). 

Such  are  the  changes  in  irritability  which  occur  in  cases  of  nerve- 
lesion  and  degeneration  of  moderate  and  considerable  degrees  of 
severity.  Certain  variations  are  occasionally  met  with,  and  these 
occur  especially  in  cases  of  neuritis  of  slight  degree.  In  severe  cases 
the  fall  in  nerve  irritability,  which  usually  commences  in  the  middle  of 
the  first  week,  may  not  take  place  until  the  end  of  this  week,  although 
it  may  then  progress  so  rapidly  that  no  response  to  stimulation  can  be 
obtained  at  the  end  of  the  second  week.  An  example  of  this  is  shown 
in  Fig.  38.*  The  same  chart,  and  some  of  those  that  follow  it,  illus- 
trate another  veiy  common  variation  from  the  type  above  described. 
There  may  be  no  fall  in  the  voltaic  excitability  of  the  muscles  before 
the  commencement  of  the  degenerative  increase.  Indeed,  this  initial 
fall  is  as  often  absent  as  present.  The  change  in  the  nutrition  of  the 
muscle  may  then  coincide  with,  instead  of  succeeding,  the  degeneration 
of  the  nerve-endings  (Figs.  38,  40).  Sometimes,  if  the  degeneration  is 
rapid,  there  is  an  interval  of  a  few  days  before  the  secondary  changes 


Fig.  38.  Fig.  39. 

WK5.   I         2        3 


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P 

NONE. 

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— 

— 

--- 

-- 

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nil 

Fig.  38. — Severe  neuritis,  paralysis  complete  for  months  j  recovery  imper- 
fect, liritability  of  nerve  normal  during  first  week;  fell  rapidly  during  the 
second ;  lost  at  its  close.  Simultaneous  increase  in  voltaic  irritability.  The 
divisions  on  the  left  side  represent  cells  of  the  voltaic  battery,  and  half- 
centimetres  of  tlie  secondary  coil  of  St5hrer*s  induction  apparatus,  in  this  and 
tlie  following  charts. 

Fig.  39.— Slight  neuritis;  slight  return  of  power  on  seventh  day,'slowly  in- 
creasing; I'.ormal  in  fifth  week.  Muscle:  faradic  irritHhih'ty  lessening  fmm 
fourth  diiy,  lost  on  fifteenth,  reappearing  on  twentieth.  Voltaic  irritability 
normal  till  twelfth  day,  then  augmented.  >erve-trunk:  lowered  from  fourth 
day  without  in  tial  increase,  reappearing  on  twenty-third  day;  changed  alike  to 
faritdism  and  voltaism. 

in  the  muscle  have  reached  the  degree  necessary  to  produce  increased 
irritability.  In  a  slight  degree  of  degeneration  the  increase  in  the 
voltaic  irritability  of  the  muscles  may  be  postponed  for  a  week  or  more 
after  the  nerve  irritability  begins  to  fall,  and  until  the  rise  occurs  the 

•  This  and  the  following  charts  are  from  cases  of  neuritis  of  the  facial  nerve. 


76 


LESIONS  OP  NERVES. 


voltaic  irritability  of  tHe  muscles  may  remain  normal,  eren  when  tlieir 
faradic  irritability  falls  with  that  of  the  nerve-tnmk.  An  instance  of 
this  is  shown  in  Fig.  39. 

When  the  nerve-lesion  is  very  slight  in  degree,  the  fall  in  nerve  irrita- 
bility may  be  preceded  by  a  rise,  which  may  be  far  greater  in  degree  than 

the  subsequent  fall.  In  slight 
cases  the  fall  may  indeed  be  not 
only  slight,  it  may  be  altogether 
absent,  so  that  the  rise  consti- 
tutes the  only  symptom.  This 
initial  increase  in  irritability  is 
important  as  the  manifestation 
of  the  slightest  degree  of  altera- 
tion in  the  nutrition  of  nerve- 

FlG.  40. — Slight  neuritis;  power  not  entirely  fibres.*  It  may  last  for  a  feW 
lost,  and  becoming  normal  during  the  ^  f  ^  j^  ^j,-  ^q. 
third  week.  Shght  deg-  react,  m  muscle  •'  >.  o  /» 
developed  during  second  week,  and  con-  and  I  have  once  known  it  to  con- 
tinued till  seventh.  Nerve  irritability  tinue  for  five  weeks  (Fig.  41). 
increased  during  second  week,  passing  .  ,, .  ,  .-,  ,  •  •  •. 
during  the  third  into  transient  depres-  Although  the  change^  m  imta- 
sion,  tlie  voltaic  irritability  falling  more  bility  of  the  nerve  is  usually 
than  the  faradic.  ^j^g   ^^^^^^  ^o    both   currents,  a 

partial  exception  to  this  rule  is  presented  by  the  increase  in  irritability 
we  are  now  considering.     The  change  is  not  always  quite  equal  to 

WKSt    2    3     4     6     6    7     6    9    «0    '»     II   1^    14  lg    »6    17    IB  '9  ^ 


A/KS. 

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Fig.  41. — Slight  neuritis;  quick  recovery  of  power  during  third  and  fourth 
weeUs.  Degen.  react,  in  muscles  present  at  the  end  of  the  second  week, 
slowly  lessening,  but  present  in  slight  degree  until  the  fourth  month. 
Considerable  increase  in  irritability  in  the  nerve-trunk  at  the  end  of  the 
sopnnd  week,  greatest  to  the  faradic  shock  (crosses),  and  lasting  until  the 
fii'th  week. 

faradism  and  to  voltaism,  and  it  is  frequently  much  more  marked  to 
the  isolated  faradic  shocks  than  it  is  to  either  the  voltaic  current  or  the 
rapid  succession  of  shocks  that  constitutes  the  faradic  current.  This 
is  shown  very  strikingly  in  Fig.  41,  and  in  less  degree  in  Fig.  43.  In 
the  former  the  irritability  to  the  two  currents  was  the  same  ;  in  the 
latter  that  to  voltaism  was  distinctly  the  greater.  I  have  once  met 
with  a  slight  but  distinct  and  prolonged  diminution  in  faradic  irrita- 
bility when  no  change  could  be  found  to  voltaism  (see  Fig.  42). 

*  A  similar  change  is  met  with  in  some  central  di^seases,  aa  chorea  and  paralysis 
agitaua. 


SYMPTOMS. 


77 


Lessened  irritability  to  faradism  with  distinct  increase  to  voltaism  has 
been  observed  in  an  ulnar  nerve,  the  seat  of  traumatic  paralysis 
(Bernhardt).  In  one  case  of  extensive  peripheral  neuritis  of  obscure 
toxic  origin,  in  which  the  face  on  both  sides  was  paralysed  for  a  time — 
a  peculiar  nutritional  change  in  the  nerves,  where  the  action  of  the 


Fig.  42. 
WEfKS  1     X 3 4^^ 5      6      7 


Fig.  43. 
DAYSx  4-    6    8    10  ft  14 


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Fig.  42. — Slight  neuritis :  power  regained  dnving  the  second  week, 
Deg.  react,  in  muscle  distinct  on  eighth  day,  lessening  during  second 
and  third  weeks.     Nerve:  lowered  fai'adic,  normal  voltaic  irritahility. 

Fig.  43. — Very  slight  neuritis.  No  change  in  muscular  irritability  ;  irri- 
tability of  nerve-trunk  increased  from  fourth  to  eighth  day,  most  to  faradic 
shock  (crosses),  least  to  far.idic  current. 

toxic  agent  was  less  than  at  their  extremities,  and  made  the  nerves  in- 
sensitive to  the  momentary  induced  current, — the  voltaic  excitability  of 
the  facial  nerves  was  not  lessened,  although  faradic  irritability  was  lost 
to  such  a  current  as  could  be  borne. 

The  electrical  excitability  of  a  nerve  and  its  power  of  conduction 
are  usually  lost  together.  But  when  a  nerve  is  recovering  from  a 
local  lesion,  and  regeneration  is  in  progress  below,  some  power  of 
conducting  the  impulse  that  is  excited  above  the  lesion  may  return  in 
the  peripheral  portion  before  it  becomes  excitable  by  any  form  of 
electricity.  This  is  true  of  the  impulse  from  the  brain,  and  that  excited 
by  electricity.  This  seems  to  show  that  the  nerve-fibres  can  conduct — 
that  is,  pass  on  the  nerve-force  they  receive  as  such,  before  they  can 
produce  nerve-energy  in  response  to  the  influence  of  a  "  stimulating  " 
force  acting  on  them.  Another  rare  anomalous  condition  has  been 
noted  by  Bernhardt  and  G-rUnberg  in  cases  of  recent  nerve  injury. 
When  the  lesion  arrests  all  conduction  of  the  voluntary  stimulus  a 
strong  faradic  stimtdation  of  the  nerve  above  the  lesion  may  still  cause 
slight  contraction  in  the  paralysed  muscles. 

Recovery  of  the  nerve  is  attended  by  gradual  restoration  of  power 
over  the  muscles,  the  nutrition  of  which  slowly  improves  ;  but  if  the 
wasting  has  been  considerable,  a  long  time  elapses  before  they  regain 
their  normal  size.  Indeed,  as  already  stated,  they  may  be  always 
somewhat  smaller  than  the  corresponding  muscles  on  the  unaffected 
side.  Curious  secondary  spasmodic  symptoms  are  common  after  palsy 
of  the  facial  nerve  (q.v.) — contractures,  spontaneous  contractions,  and 
associated  over-action  of  different  muscles.  The  last  is  very  rarely  met 
with  after  palsy  of  the  nerves  of  the  limbs,  but  coarse,  fibrillary. 


78  LESIONS   OF   NEHVES. 

muscular  contractions,  and  a  tendency  to  cramp,  are  not  uncommon, 

and  may  persist  for  years.     (See  also  Neuritis.) 

Sensory  symptoms  also  result  from  lesions  of  the  nerves  that  contain 
sensory  fibres.  The  interruption  of  the  fibres  arrests  conduction,  and 
causes  loss  of  sensation  in  the  part  supplied  by  the  nerve,  just  as  it 
causes  motor  palsy.  But  a  slight  lesion  of  a  mixed  nerve  may  cause 
persistent  muscular  paralysis,  and  sensation  be  unaffected,  or  impaired 
only  in  slight  degree  and  for  a  short  time.  This  restdt  is  so  frequent 
that  we  cannot  ascribe  it  to  a  dilference  in  the  relative  damage  to  the 
motor  and  sensory  fibres.  It  must  be  due  either  to  the  fact  that  the 
sensory  fibres  recover  more  readily,  or  that  a  slight  degree  of  conduction 
may  suffice  for  the  stimulation  of  the  sensory  centres  in  the  brain,  and 
not  for  that  of  the  muscular  fibres.  There  is  also  a  third  hypothesis, 
and  that  is  a  difference  in  the  sensory  and  motor  impulses  in  physio- 
logical character.*  In  another  class  of  cases,  however,  we  must  seek 
a  different  explanation  of  the  persistence  of  sensibility  in  the  area 
supplied  by  the  nerve.  There  may  be  no  loss  even  when  a  nerve  is 
completely  divided.  This  is  observed  chiefly  near  the  extremity  of  a 
limb.  It  can  only  be  explained  by  what  is  termed  "  recurrent  sen- 
sibility." Anastomoses  exist  between  the  terminal  fibres  of  different 
nerve-trunks,  and  it  would  seem  that  this  contiguity  of  sensory  fibres 
suffices  for  conduction.  Individual  differences  are  seen  to  exist  in  this. 
Thus,  of  two  persons  in  whom  the  ulnar  nerve  at  the  wrist  has  been 
divided,  one  may  have  total  anaesthesia  in  the  fingers  supplied  by  the 
nerve,  and  in  the  other  there  may  be  little  or  no  loss. 

The  peripheral  segments  of  the  sensory  fibres  degenerate,  like  the 
motor  nerves.  We  cannot  test  them,  and  ascertain  the  degeneration 
in  the  same  way,  at  least  in  severe  lesions,  because  their  stimulation 
cannot  be  perceived.  Nevertheless  an  altered  polar  reaction  has  been 
observed  in  slight  cases,  analogous  to  that  which  occurs  in  the 
muscles.  A  sensation  is  produced  with  the  positive  voltaic  pole  by 
a  weaker  cm-rent  than  is  required  with  the  negative  pole.  In  some 
cases  of  nerve  injury  and  neuritis  a  delay  in  conduction  of  pain  may  be 
observed. 

Increased  and  disordered  sensitiveness  in  the  area  of  distribution  of 
the  nerve,  with  or  without  spontaneous  pain,  is  very  common  in  cases 
of  partial  lesions  of  nerves.  It  must  be  referred  to  the  morbid  changes 
in  their  fibres,  alterations  in  the  nutrition  of  the  axis-cylinder  or  nerve- 
endings,  probably  analogous  to  the  slight  changes  in  the  motor  nerves 
which  increase  their  excitability.  Pain  and  tenderness  of  the  nerve- 
trunk  are  also  frequent  in  the  same  cases,  due  no  doubt  to  the  increased 
sensitiveness  of  the  "  nervi  nervorum  "  distributed  in  the  sheath.  The 
sheath  of  each  nerve  is  part  of  the  dis+  ribution  of  its  own  fibres,  and 
those  that  end  in  the  sheath  seem  lu  be  very  readUy  deranged.  We 
shall  see  the  importance  of  this  consideration  in  relation  to  neuritis  and 

*  Tlie  experiments  of  Lenderitz  ('  Zeitschr.  f.  klin.  Med.,'  Bd.  iii)  merely  confirm 
the  observed  fact,  and  do  not  really  explain  it,  as  he  and  others  have  assumed. 


SYMPTOMS,  79 

neuralgia.  The  pains,  botli  local  and  distant,  are  sometimes  very 
severe  and  persistent. 

Reflex  Action.-'— AR  lesions  of  nerves  abolish  reflex  action  in  the  area 
of  the  distribution  of  the  nerve  affected,  except  those  that  involve 
so  shght  a  change  as  to  cause  increased  sensitiveness,  Mjotatic  irri- 
tability is  also  lost,  f lom  damage  to  either  the  motor  or  sensoiy- fibres 
concerned,  and  this  loss  may  persist  long  after  the  other  symptoms. 

Vaso-motor  and  trophic  disturbance  is  a  common  consequence  of 
lesions  of  nerves.  It  is  most  severe  in  those  that  are  inflammatory  in 
part  or  altogether.  The  vaso-motor  nerves  run  in  the  mixed  nerve- 
trunks,  and  suffer  with  the  other  fibres.  The  general  character  of  the 
disturbance  has  already  been  described  (p.  27).  When  the  lesion  is 
acute  and  irritative,  vascular  dilatation  and  an  increase  of  temperatiu-e 
occur  at  first,  and  are  followed  by  passive  hyperaemia  and  lowered  tem- 
perature. There  may  be  oedema,  and  an  increased  secretion  of  sweat. 
The  changes  in  the  skin,  acute  and  chronic,  are  those  that  have  been 
already  described.  "  G-lossy  skin,"  red,  smooth,  and  thin,  is  especially 
common,  and  is  frequently  accompanied  by  atrophy  of  the  subcutaneous 
tissue,  and  pei-haps  of  the  bone,  so  that  the  finger-tips  become  narrow, 
and  present  an  aspect  that  is  very  characteristic.  Burning  pain,  with 
tenderness,  often  accompanies  the  change,  and  may  continue  for  long. 
Occasionally,  instead  of  becoming  thin,  the  epidermis  increases  in 
thickness,  the  nails  often  become  thick  and  rough,  and  present  trans- 
verse or  longitudinal  furrows.*  In  parts  where  there  is  much  sub- 
cutaneous tissue  this  may  also  become  thickened  and  have  a  peculiar 
doughy  feel,  resembling  that  met  with  in  myxoedema.  The  growth  of 
the  hair  may  be  lessened  or  increased.  Sloughing  of  the  skin  is  far 
less  common  than  in  central  diseases,  but  eruptions,  particularly  of 
the  nature  of  erythema,  eczema,  herpes  and  pemphigus,  may  also  be 
met  with.  Vesication  occurs  with  extreme  readiness,  and  sometimes 
vesicles  or  bullae  appear  to  form  spontaneously.  Ulcers  result  which 
erode  and  destroy  the  tissues,  especially  of  the  finger-ends.  Mustard 
plasters  also  blister  the  skin  more  readily  than  in  health.  It  is  im- 
portant to  remember  these  facts,  because  the  affection  is  one  in  which 
hot  applications  are  often  recommended.  A  gentleman  dislocated  his 
shouldei-,  and  either  by  the  displaced  bone,  or  in  the  reduction,  the 
brachial  plexus  was  seriously  injured.  The  muscles  of  the  hand 
wasted,  and  the  skin  became  glossy.  He  was  advised  by  a  surgeon  to 
bathe  the  hand  daily  in  hot  water.  One  day  his  wife  bathed  it  in 
water  which  to  her  was  pleasantly  warm,  and  which  caused  him  no 
discomfort,  but  the  result  was  that  the  hand  was  covered  with  bhsters, 
and  ulcers  were  left  which  did  not  heal  for  months. 

*  Several  excellent  illustrations  of  these  changes  iire  given  by  BowUiy,  loc.  cit., 
where  also  a  full  account  of  traumatic  lesions  is  given.  The  observations  of  Beru- 
harlt  ('Viichow's  Archiv,'  1886)  appear  to  show  that  the  retarded  growth  of  the 
nails  is  independent  of  the  influence  of  the  particular  nerves.  Great  individual 
Tariatioiis  are  found  with  identical  lesions. 


80  COMPRESSION. 

The  joints  often  suffer  in  their  nutrition.  Acute  inflammation,  such 
as  occurs  sometimes  in  spinal  affections,  is  rare,  but  chronic  changes  in 
the  joints  are  frequent,  with  much  tenderness;  alterations  in  the 
articular  surfaces,  and  fibrous  adhesions  result,  which  limit  movement. 
When  the  muscular  wasting  is  slight,  the  condition  may  resemble  a  pri- 
mary .joint  affection,  and  this  mistake  in  diagnosis  is  sometimes  actually 
made. 


INJURIES  OF  NERVES:    COMPRESSION. 

Traumatic  lesions  of  nerves  are  in  the  province  of  surgery.  They 
belong  to  the  physician  only  in  so  far  as  their  effects  are  produced  by 
the  agency  of  a  neuritis,  the  mechanical  origin  of  which  may  escape 
notice.  Neuritis  is  described  in  the  next  section.  A  word  is  needed, 
however,  on  the  subject  of  simple,  slow  compression  of  nerves,  not 
because  the  symptoms  are  grave,  but  because  they  often  give  rise  to 
very  grave  mental  concern  on  the  part  of  the  patient,  and  to  occa- 
sional doubts  in  the  mind  of  his  medical  attendant.  Compression  of 
a  nerve  may  be  sudden  or  slow.  Sudden  compression  bruises  the 
nerve-fibres,  prodacing  definite  lesions,  and  also  sets  up  neuritis. 
Symptoms  often  occur  too  soon  to  be  explicable  as  neuritic.  A  slight 
degree  of  these  is  familiar  as  a  result  of  pressure  on  the  sciatic  nerve  in 
the  act  of  sitting.  There  is  first  tingling  in  the  region  of  distribution 
of  its  fibres,  often  with  a  sense  of  warmth,  with  disordered  sensations 
and  even  a  loss  of  sensibility  in  the  sMn.  Often  this  is  accompanied 
by  an  inability  properly  to  put  in  action  the  muscles  that  are  supplied 
by  the  nerve.  All  the  symptoms  pass  away  in  a  few  minutes,  a  sense 
of  cold  and  an  inability  to  direct  movements  being  often  the  last 
effects.  If  they  do  not  pass  away,  the  persistent  effects  are  ascribed  to 
a  neuritis  such  as  is  produced  by  any  other  cause. 

But  neuritis  is  not  the  primary  lesion,  and  the  initial  symptoms 
are  not  produced  by  the  agency  of  inflammation.  Hence,  when 
they  persist,  it  is  doubtful  whether  we  should  ascribe  them  to  the 
inflammation  unless  they  occur  after  an  interval.  The  mechanism  by 
which  the  tingling  and  early  anaesthesia  are  produced,  probably  causes 
also  the  lasting  symptoms.  The  compression  no  doubt  causes  anaemia, 
but  the  known  sensitiveness  of  nerves  to  mechanical  stimulation  makes 
it  probable  that  the  effects  are  chiefly  produced  by  the  mechanical 
influence  of  the  pressure  on  the  elements  of  the  nerve-fibres,  and  that 
a  compression  for  a  few  hours  has  such  an  effect  in  separating  the 
molecular  elements  of  the  white  substance,  as  to  set  up  a  secondarv 
defeneration  of  the  same  character  as  thai  which  results  from  division 
of  a  nerve.  The  neuritis  is  thus  no  more  the  cause  of  symptoms  in 
compression  than  in  division.  In  each  there  is  a  displacement  of 
molecules  interrupting  conduction.  This  is  also  the  conclusion  of 
Weir  Mitchell,  who  found  that  a  pressure  of  eighteen  to  twenty  inches 


COMPRESSION.  81 

of  mercmy  for  twenty  seconds  abolislied  tlie  power  of  conduction, 
though  only  for  a  few  minutes,  in  spite  of  conspicuous  change  in  the 
white  substance.  The  latter  is  still  greater,  and  the  effects  more 
enduring,  in  a  contusion. 

The  cause  of  the  tingling  in  the  region  of  distribution  of  the  sciatic 
nerve  is,  as  already  said,  readily  recognised  when  it  is  due  to  pressure 
by  sitting.  But  it  is  otherwise  when  the  pressure  is  on  the  nerves  of 
the  arm  and  is  produced  during  sleep.  The  tingling  may  be  felt  in 
the  region  of  the  ulnar  nerve,  in  consequence  of  flexion  of  the  elbow, 
or  in  the  whole  hand,  from  the  pressure  on  the  nerves  of  the  brachial 
plexus  on  the  side  on  which  the  patient  is  lying.  Waking  up  with  the 
tingling,  and  sometimes  with  cramp  or  powerlessness  in  the  hand,  he  is 
naturally  alarmed.  The  diagnosis,  however,  is  rendered  easy  by  the 
unilateral  character  of  the  symptoms,  and  by  the  fact  that  they  are 
always  felt  on  the  side  that  is  bearing  the  weight  of  the  body.  There 
may,  however,  be  great  dif&culty  in  diagnosing  this  form  of  compression 
from  actual  contusions.  The  symptoms  are  produced  much  more 
readily  when  the  general  health  is  bad;  they  last  longer,  and  more 
often  end  in  neuritis. 

Gradual  compression  may  result  from  continuous  pressure,  such  as 
that  of  a  crutch  or  the  growth  of  an  adjacent  tumour,  or  from  the 
implication  of  the  nerve  in  a  cicatrix  or  callus.  Of  new  growths  com- 
pressing nerves,  exostoses  and  aneurisms  are  most  frequent.  The 
symptoms  of  this  form  approach  those  of  neuritis ;  they  may  be 
sensory,  motor  or  trophic.  They  are  ushered  in  by  hyperassthesia, 
which  is  followed  by  anaesthesia,  seldom  complete  in  degree  or  quality, 
tactile  and  muscular  sensibility  being  usually  retained  when  other 
forms  are  lost.  Intense  and  persistent  hypersesthesia  or  complete 
anaesthesia  point  to  neuritis.  Motor  weakness  and  trophic  changes 
appear;  there  may  be  atrophy  or  hypertrophy  of  the  nails,  trophic 
ulcers,  or  joint  affections.  Among  rarer  symptoms  are  local  spasm 
with  fibrillary  twitchings  and  reflex  convulsions.  In  simple  com- 
pression the  symptoms,  as  a  rule,  disappear  after  the  removal  of  the' 
cause;  the  subjective  sensations  are  the  first  to  vanish,  then  the 
anaesthesia  goes,  and  lastly — in  severe  cases  not  till  after  some  months 
— ^the  motor  disturbances. 

In  rapid  compression  there  may  be,  according  to  the  duration  of  the 
injury,  merely  congestion  at  the  affected  spot  or  peripheral  degeneration 
starting  from  it.  A  nerve  which  has  been  long  compressed  is  narrow 
at  the  affected  part,  where  it  may  even  be  flattened  into  a  ribbon ; 
above  and  below  it  is  normal  or  swollen.  The  compressed  portion 
may  be  white  from  anaemia  or  red  from  congestion.  Eventually  degene- 
rative or  neuritic  changes  may  develop,  and  the  former  are  sometimes 
found  above  the  lesion  as  well  as  below. 

Treatment  is  essentially  surgical,  but  the  physician  must  aim  at 
maintaining  the  nutrition  of  the  muscles  by  the  usual  means  during 
the  recovery  of  the  nerve,  when  this  can  be  released  from  compression. 

VOL.   I.  6 


82  NEUiilTlS. 


INFLAMMATION   OF   NERVES:    NEURITIS. 

Neuritis,  or  inflammation  of  nerves,  presents  various  cliaracters, 
which  have  led  to  the  distinction  of  numerous  forms.  The  inflam- 
mation of  single  nerves  usually  begins  in  the  outer  sheath  of  the  nerve, 
and  this  has  been  distinguished  as  "perineuritis."  It  may  involve 
also  or  chiefly  the  connective  tissue  between  the  bundles  of  nerve-fibres 
("interstitial  neuritis"),  or  the  nerve-fibres  themselves  ("parenchy- 
matous or  degenerative  neuritis").  Some  forms  of  neuritis  have  a 
tendency  to  ascend  the  nerve,  and  this  variety  has  been  termed 
**  ascending  neuritis  "  or  "  neuritis  migrans."  One  nerve-trunk  may 
be  primarily  affected,  or  many  nerves  may  suffer  at  the  same  time. 
The  latter  condition,  "  primary  multiple  neuritis,"  is  of  great  import- 
ance, and  vdll  be  described  in  a  separate  section. 

Most  forms  of  neuritis  may  be  either  acute  or  chronic.  Acute 
neuritis  usually  subsides  into  a  chronic  stage,  and  its  symptoms  may 
thus  persist  for  a  long  time.  Other  varieties  that  have  been  distin- 
guished depend  upon  the  cause  to  which  the  inflammation  is  secondary. 
Thus  we  have  cancerous,  syphilitic,  gouty,  diabetic,  and  other  forms. 

Causes. — Injury  causes  more  or  less  inflammation,  which  occurs 
readily,  and  follows  very  slight  damage.  Neuritis  may  thus  be  set 
up  by  all  sorts  of  wounds,  by  contusions,  by  compression  from  with- 
out or  by  adjacent  structures,  and  by  over-extension  of  the  nerves. 
Those  nerves  that  pass  by  joints  are  liable  to  injury  from  dislocations, 
either  by  the  displacement  of  the  bones,  or  during  reduction.  In 
fractures  the  nerves  m.ay  be  injured  directly,  or  may  be  compressed 
by  the  callus  that  is  formed.  Nerves  are  sometimes  damaged  by  a 
violent  contraction  of  the  muscles  through  which  they  pass.  It  is 
probable  also  that  muscular  effort  excites  neuritis  in  other  situations, 
especially  in  persons  who  are  predisposed.  Moreover,  a  strain  on  the 
fibrous  tissues  of  joints,  fasciae,  &c.,  may  set  up  inflammation  in  them, 
which  may  spread  to  the  nerves. 

Neuritis  may  arise  by  extension  from  adjacent  inflammation;  this 
is  particularly  seen  in  the  case  of  phlebitis.  The  nerves  near  suppu- 
rating joints  may  be  involved,  and  even,  it  is  said,  those  that  pass  by 
a  joint  the  seat  of  simple  inflammation.  The  latter  is  certainly  rare. 
The  intercostal  nerves  have  been  affected  by  extension  from  an  in- 
flamed pleura.  The  cranial  nerves  and  the  spinal  nerve-roots  are 
involved  by  extension  from  the  membranes. 

Exposure  to  cold  is  another  cause  of  neuritis.  It  is  then  often 
called  "  rheumatic."  The  nerve,  or  rather  its  sheath,  may  be  primarily 
affected,  or  the  first  effect  may  be  inflammation  of  the  fasciae,  from 
which  the  inflammation  spreads  to  the  nerve-sheath.  Persons  who 
are  liable  to  muscular  rheumatism,  and  those  who  are  gouty,  suffer 


PATHOLOGiOAL  ANATOMY. 


83 


thus  with  especial  frequency.  But  exposure  to  cold  and  the  toxins  of 
gout  and  rheumatism  also  act  in  another  way,  by  causing  a  blood-state 
which  acts  on  many  nerves  at  the  same  time.  Many  general  diseases 
have  a  similar  power  of  causing  either  an  isolated  or  a  multiple 
neuritis.  It  is  through  this  agency  that  diphtheritic  paralysis  is 
produced,  and  the  same  effect  may  be  due  to  the  presence  of  other 
toxic  material  in  the  blood,  metallic  poisons,  alcohol,  &c.,  an  account 
of  which  will  be  found  in  the  section  on  Multiple  Neuritis.  When 
an  isolated  neuritis  occurs  during  an  acute  disease  it  is  probably 
produced  through  the  agency  of  compression,  unnoticed  during  the 
prostration.  Neuritis  has  also  been  found  in  the  vicinity  of  bedsores 
(Pitres),  and  though  probably  due  to  pressure  may  be  part  of  the 
mechanism  by  which  they  are  caused.  Some  chronic  general  diseases 
cause  only  isolated  neuritis, — either  simple,  as  that  which  is  due  to 
gout  (when  the  general  disease  must  be  regarded  chiefly  as  a  pre- 
disponent) ;  or  special,  as  syphilitic,  cancerous,  or  leucocythsemic 
neuritis,  in  which  there  is  an  infiltration  of  the  nerve  with  the  special 
tissue-element  characteristic  of  the  general  disease. 

The  characters  of  the  simple  isolated  form  constitute  the  chief 
subject  of  this  section. 

PathologioaIi  Anatomy. — The  changes  differ  according  as  the 
inflammation  affects  primarily  the  adventitial  connective  tissue, 
"adventitial  neuritis,"  or  the  fibres  themselves  in  the  "paren- 
chymatous" form.  Isolated  neuritis  is  generally  adventitial,  and 
wiQ  be  first  described.  In  acute 
inflammation  the  affected  part  of 
the  nerve  is  red,  softened,  and 
swollen.  The  redness  depends 
on  distended  vessels,  which  may 
be  visible  on  the  surface;  in 
more  acute  cases  it  may  result 
from  the  presence  of  minute 
haemorrhages.  The  swelling  is 
due  to  oedema,  or  to  a  sero- 
fibrinous exudation,  sometimes 
jelly-like  in  aspect.  The  micro- 
scope shows  leucocyte-like  cor- 
puscles surrounding  the  vessels, 
infiltrating  the  sheath  (Fig.  44) 
and  acc»'iiulating  between  it  and 
the  nerve.  There  may  be  even 
email  extravasations  of  blood. 
Suppuration  is  extremely  rare.  These  changes  may  be  limited  to  the 
sheath  in  what  is  called  "perineuritis,"  or  may  extend  into  the 
substance  of  the  nerve  in  "  interstitial  neuritis."  In  the  latter  case 
the  lymphoid  corpuscles  infiltrate  the  septa,  and  may  even  be  seen  in 
the  substance  of  the  f  ascicidi,  between  the  nerve-fibres.    These  changes 


Fig.  44. — Neuritis :  degeneration  of  nerve- 
fibres,  the  myelin  broken  up  into  masses, 
globules,  and  granules.  Accumulation  of 
leucocytes  in  uerve-sheath.  P'roin  a  case 
of  multiple  neuritis.     (After  Ley  den.) 


84  NEURITIS. 

may  be  continuous  along  a  considerable  tract  of  tbe  nerve  (diffuse 
neuritis),  but  more  frequently  they  are  chiefly  marked  at  certain 
places,  which  are  separated  by  portions  of  the  nerve  which  are  so 
little  affected  as  to  appear  to  the  naked  eye  to  be  normal  ("focal" 
or  "disseminated  neuritis").  The  foci  of  inflammation  are  chiefly 
situated  at  places  where  the  nerve  turns  round  a  bone,  or  emerges 
from  canals  or  fasciae,  or  divides. 

The  extent  to  which  the  nerve-fibres  suffer  varies  much.  They 
usually  present  little  change  when  the  inflammation  is  limited  to 
the  sheath,  unless  the  nerve  lies  in  a  bony  canal,  or  in  rigid  fibrous 
tissue,  within  which  the  sheath  cannot  expand ;  its  swelling  then  exerts 
pressure  on  the  fibres.  When  the  inflammation  is  interstitial  the 
fibres  suffer  more  readily,  although  not  invariably.  On  the  other 
hand,  they  are  sometimes  found  much  altered,  when  the  connective- 
tissue  elements  are  but  little  affected.  In  this  case  the  inflammation 
is  "  parenchymatous  "  and  begins  in  the  nerve-fibres,  the  interstitial 
tissue  being  secondarily  involved  or  unaffected.  The  changes  in  the 
fibres  are  almost  the  same  as  in  degeneration  (Figs.  33  and  34). 
It  was  pointed  out  how  closely  the  two  processes  of  inflammation 
and  degeneration  are  connected,  and  how  difficult  it  is  to  separate, 
or  even  to  distinguish  them,  in  many  cases.  The  myelin  of  the  white 
substance  first  breaks  up  into  segments,  more  or  less  elongated,  often 
with  smaller  globules  of  myelin  between  or  beside  them.  The  masses 
are  cloudy  or  finely  granular  in  aspect.  The  axis-cylinders  are  inter- 
rupted where  the  myelin  is  divided.  The  nuclei  of  the  sheath  are 
increased  in  number,  and  the  protoplasm  around  them  is  increased  in 
quantity.  Next  the  myelin  is  divided  into  smaller  globules  and 
granules,  and  the  axis-cylinder  is  no  longer  recognisable.  The  myelin 
then  disappears  in  parts  of  the  tubes,  while  it  remains  at  other  parts, 
but  is  still  more  finely  divided.  Lastly,  the  sheaths  become  empty 
and  very  narrow,  containing  only  nuclei  at  intervals,  with  here  and 
there  a  little  finely  granular  material,  or  sometimes  some  brownish 
pigment  granules.  The  process  may  thus  go  on  to  complete  destruc- 
tion of  the  fibres.  These  commonly  suffer  unequally;  fibres  that 
have  a  normal  appearance  are  scattered  among  those  that  are  much 
altered. 

In  chronic  interstitial  neuritis  the  axis-cylinders  often  suffer  less 
than  the  white  substance.  The  latter  becomes  atrophied,  so  that  the 
fibres  are  smaller  than  normal.  If  the  interstitial  inflammation  is  very 
intense  the  fibres  may  break  up  as  above  described.  As  the  inflam- 
mation subsides,  the  new  cellular  elements  that  have  made  their 
appearance  assume  the  aspect  of  fusiform  cells,  and  fibrous  tissue  is 
developed,  either  from  these  eel';,  which  become  less  numerous,  or 
from  the  intercellular  exudation.  This  fibrous  tissue  surrounds  and 
encloses  the  fasciculi,  a  condition  that  has  been  termed  "  sclerosis  of 
the  nerve."  A  firm  fusiform  swelling  may  remain  at  the  affected  spot, 
and  this  may  be  adherent  to  adjacent  structures.     The  axis-cylinders 


SYMPTOMS.  85 

are  unequally  affected.  Those  which  are  involved  undergo  typical 
degeneration.  Sometimes  fat  is  ultimately  formed  in  the  new  con- 
nective tissue,  a  condition  that  has  been  rather  unnecessarily  termed 
"  lipomatous  neuritis  "  (Leyden)  ;  the  fatty  deposit  does  not  seem  to 
be  related  to  any  special  form  of  neuritis.  Occasionally  the  connective 
tissue  overgrowth  is  so  extensive  that  the  whole  nerve  is  converted 
into  a  sclerotic  cord  (Virchow's  "  proliferative  "  form). 

In  syphilitic  neuritis,  which  affects  chiefly  the  cranial  nerves,  there 
is  a  cellular  growth  in  the  sheath  and  interstitial  tissue  similar  to  that 
which  constitutes  other  syphilitic  new  formations,  with  a  variable 
amount  of  alteration  of  simpler  inflammatory  character.  The  growth 
in  the  sheath  may  amount  to  a  distinct  syphilitic  tumour,  with  or 
without  interstitial  changes.  In  cancer,  nerves  adjacent  to  the  new 
growth  may  present  simple  interstitial  neuritis,  or  may  be  infiltrated 
by  a  gi'owth  of  cancer-elements  spreading  to  the  nerve  by  direct 
extension.  In  leucocythsemia  there  is  a  dense  infiltration  of  the  nerve 
with  leucocytes. 

The  destructive  changes  in  the  nerve-fibres  extend  down  the  nerve 
to  the  periphery  in  a  process  of  secondary  degeneration,  more  or  less 
inflammatory  in  nature,  as  already  described  in  secondary  degeneration. 
Usually  the  central  portion  of  the  nerve  remains  free,  the  alterations 
ceasing  a  short  distance  above  the  seat  of  the  primary  inflammation. 
In  rare  cases  an  ascending  neuritis  (n.  migrans)  passes  up  the  nerve, 
and  may  spread,  at  a  plexus,  to  other  nerve-bundles  and  other  nerve- 
trunks.  The  formation  of  fibrous  tissue  in  the  inflamed  part,  and 
the  cicatricial  contraction  of  this  tissue,  often  prolong  the  irritation 
of  the  nerve-fibres  and  the  symptoms  due  to  this,  and  a  state  of 
"  chronic  neuritis  "  is  said  to  be  left. 

Symptoms. — The  symptoms  of  neuritis  vary  extremely  according  to 
its  intensity,  its  extent,  and  the  nerve  that  is  affected.  In  three  places, 
the  brachial  plexus,  the  sciatic  nerve,  and  the  crural  nerves,  the  sym- 
ptoms are  sufficiently  special  to  make  their  separate  description 
desirable,  although  the  general  account  applies  to  them  also. 

The  onset  of  the  acute  form  is  sometimes  attended  by  some  con- 
stitutional disturbance,  especially  when  many  nerves  are  affected. 
The  chief  symptoms  are  local.  The  most  prominent  is  pain,  felt  in 
the  inflamed  part  of  the  nerve,  and  also  often  in  the  part  to  which 'it 
is  distributed  (see  p.  78).  Sometimes  the  pain  involves  the  whole 
limb,  and  in  severe  cases  it  may  be  most  intense,  burning,  boring,  rarely 
darting,  in  character ;  it  is  usually  worse  at  night  and  increased  by 
movement,  by  postures  that  involve  tension  or  pressure  on  the  nerve, 
and  by  whatever  causes  passive  congestion  of  the  limb,  such  as  the 
act  of  coughing.  Sometimes  it  seems  to  radiate  into  distant  parts, 
and  not  rarely  pain  is  also  felt  in  thb  corresponding  region  of  the 
opposite  limb.  The  sensitiveness  of  the  whole  of  the  affected  region 
is  increased,  and  even  the  bone  may  be  tender,  so  that  at  fii-st  attention 
may  not  be  directed  to  the  nerve,  but  when  this  is  pressed  great  pain 


86  NEURITIS. 

is  always  produced.  In  slighter  cases  tlie  pain  is  usually  limited  to 
tlie  nerve  and  its  distribution.  It  may,  indeed,  be  absent  when  the 
nerve  or  branch  is  chiefly  motor,  a  fact  difficult  to  explain  unless  the 
sheaths  of  such  nerves  contain  fewer  sensory  fibres  than  usual.  If 
the  nerve  is  accessible  to  direct  examination  it  may  be  felt  to  be 
distinctly  swollen  at  the  affected  part.  Occasionally  the  sMn  over  it 
has  been  observed  to  be  red,  and  rarely  there  has  been  slight  oedema. 
Spontaneous  sensations  may  be  felt  in  the  region  supplied  by  the 
nerve,  tingling,  &c.,  and  the  sldn  may  be  hypersesthetic.  After  a  time, 
as  the  nerve-fibres  suffer,  sensation  may  be  perverted,  or  even  lessened ; 
complete  anaesthesia  is  usually  confined  to  small  areas.  The  muscles 
supplied  by  the  nerve  become  weak  in  various  degree,  tender,  and 
present  fibrillar  twitchings ;  they  are  seldom  powerless,  but  pain  may 
prevent  their  contraction.  They  present  the  altered  electrical  reac- 
tions characteristic  of  nerve-lesions.  Increased  perspiration  has  been 
observed  in  the  part  of  the  skin  supplied  by  the  inflamed  nerve,  very 
rarely  it  is  arrested,  and  sometimes,  although  rarely,  eruptions  occur. 
Herpes  is  not  common  from  ordinary  acute  neuritis.  The  epidermis 
may  become  thickened  into  a  state  like  ichthyosis,  or  may  atrophy  and 
appear  as  "  glossy  skin."  Effusion  into  joints  has  been  observed  in 
very  rare  cases.  The  constitutional  disturbance  which  may  attend  the 
onset  subsides  ia  the  course  of  a  few  days,  but  the  pain  and  other 
symptoms  usually  persist  in  undiminished  severity  for  several  weeks. 
They  ultimately  slowly  subside  into  a  chronic  stage,  and  may  continue 
as  a  secondary  neuralgia  due  to  a  habit  of  over-action  set  up  in  the 
centre. 

In  the  chronic  form  constitutional  disturbance  is  absent,  and  pain 
is  the  prominent  symptom  from  the  first.  The  affection  of  motion  and 
sensibility,  and  the  trophic  changes,  are  similar  to  those  met  with  in 
the  acute  form.  In  both  forms  the  muscles  may  waste,  and  present 
the  reaction  of  degeneration  when  they  are  tested  with  electricity 
(p.  73).  In  slight  cases  the  initial  increase  of  excitability  in  the  nerve 
is  often  well  marked.  Trophic  changes  in  the  skin  are  very  common ; 
so  is  alteration  in  the  nutrition  of  the  joints,  and  adhesions  form, 
limiting  the  movement,  and  fixing  the  parts  in  the  position  corre- 
sponding to  the  muscular  inaction. 

When  neuritis  ascends  a  nerve,  the  symptoms  gradually  extend  in 
area ;  and  if  it  reaches  the  plexus  from  which  the  nerve  proceeds,  they 
may  extend  to  most  or  all  the  nerves  of  the  limb.  This  "  ascending 
neuritis"  is  not  rare  in  man,  may  even  reach  the  spinal  canal, 
and  there  produce  various  disturbances.  The  inflammation  may 
spread  in  the  tissue  outside  the  dura  mater,  or  may  extend  to 
the  cord,  and  cause  subacute  or  chronic  myelitis  with  or  without 
meningitis.  The  paralysis  that  occurs  secondarily  to  some  visceral 
diseases,  as  those  of  the  bladder,  and  commonly  regarded  as  reflex 
paralysis,  is  probably  produced  through  the  agency  of  an. ascending 
neuritis.     Lastly,  the  inflammation  maj    -^xtend  to  the  nerves  of  the 


DIAGNOSIS — PROGNOSIS.  87 

other  side,  usually  to  those  that  correspond  to  the  primary  seat  of  the 
disease.  Such  extension  may  be  through  the  spinal  cord  or  the 
membranes,  but  in  some  cases  symptoms  occur  in  the  nerve  of  the 
opposite  side,  without  any  indication  of  an  affection  of  the  centres. 
Experiments  on  animals  have  demonstrated  that  such  implication  of 
the  opposite  nerve  may  occur  when  the  centres  are  unaffected.  It  has 
been  called  "  sympathetic  neuritis."  We  have  seen  that  reflected 
pains  are  occasionally  felt  in  the  corresponding  nerve  on  the  opposite 
side,  and  it  is  probable  that,  as  in  neuralgia,  vaso-motor  disturbance 
in  the  nerve- sheath  may  accompany  such  pains,  and,  in  a  predisposed 
person,  may  cause  actual  neuritis. 

The  Duration  and  Course  of  neuritis  vary  much.  A  slight  acute 
neuritis  may  run  its  course  in  a  few  weeks  and  then  subside.  Much 
more  commonly  the  affection  persists  in  a  chronic  stage  for  many 
weeks,  or  even  months,  and  slowly  passes  off.  The  rheumatic  and 
gouty  forms  are  as  a  rule  much  more  tedious  than  those  that  result 
from  injury,  although  traumatic  neuritis  is  sometimes  very  insidious 
and  may  last  a  long  time  ;  it  may  ascend  the  nerve  from  its  starting- 
place  and  become  localised  in  certain  situations,  where  it  may  develop 
afresh,  and  give  rise  to  symptoms  that  often  seem  to  be  independent 
of  their  cause.  In  the  predisposed,  it  can  set  up  troublesome  local 
functional  disturbance,  hysterical  contracture,  and  the  like. 

DiAauosis. — The  diagnosis  of  neuritis  depends,  first  on  the  localisa- 
tion of  the  symptoms  to  the  distribution  of  a  certain  nerve-trunk,  and 
secondly  on  the  pain  and  tenderness  in  the  nerve.  The  diffuse  pains 
that  attend  the  onset  may  be  readily  mistaken  for  the  pains  of  acute 
rheumatism,  or  for  those  due  to  an  inflammation  of  the  bone,  but  in  the 
course  of  a  day  or  two  the  localisation  of  the  symptoms  declares  their 
nature.  The  chronic  form  is  easily  and  often  mistaken  for  neui'algia, 
and  the  diagnosis  is  the  more  difficult  because  many  so-called  neu- 
ralgias are  really  due  to  neuritis.  The  distinction  can  only  be  fully 
discussed  when  we  have  considered  the  symptoms  of  neuralgia,  but  it 
rests  chiefly  on  the  fact  that  in  neuralgia  the  pain  intermits  more 
completely  than  in  neuritis,  there  is  not  the  same  initial  tenderness  in 
the  nerve-trunks,  and  the  tender  spots  have  a  more  uniform  localisation. 
Lessened  sensibility  or  change  in  the  muscles,  showing  organic  damage 
to  the  nerve-fibres,  are  conclusive  evidence  of  neuritis.  The  pains  in 
some  central  diseases,  chiefly  those  of  the  spinal  cord,  may  be  thought 
to  be  due  to  neuritis,  but  there  is  not  the  local  tenderness  of  the 
nerve- trunk,  and  the  pain  is  not  limited  to  the  distribution  of  a  single 
nerve.  As  we  shall  see  presently,  the  diagnosis  of  multiple  neuritis 
from  affections  of  the  cord  is  far  more  difficult. 

Peosnosis. — The  gravest  form  of  simple  neuritis  is  that  in  which  the 
nerve  is  affected  secondarily  to  a  local  suppurative  inflammation.  The 
prognosis  is  best,  as  a  rule,  in  traumatic  neuritis,  but  the  rule  is  one 
to  which  the  exceptions  are  occasionally  very  considerable  in  degree. 
A  neuropathic  disposition  makes  the  prognosis  worse.     In  all  cases 


88  NEURITIS. 

the  intensity  of  tlie  symptoms,  and  tlie  evidence  of  descending  degene- 
ration, furnish  a  more  trustworthy  guide  to  prognosis  than  does  the 
mere  form  of  the  affection.  The  effects  of  complete  degeneration 
always  endure  for  some  months.  Eegeneration  does  not  occur  until 
the  original  cause  has  ceased  to  act,  and  then  occupies  many  weeks  in 
its  progress  to  the  restoration  of  functional  competence.  In  all  forms 
pain  is  apt  to  linger  on,  and  the  longer  the  older  the  patient  is. 

Treatment. — The  first  consideration  in  the  treatment  of  neuritis  is 
the  removal  of  its  cause,  if  this  can  be  discovered.  A  wound  or  injury 
or  local  inflammation  adjacent  to  the  nerve  must  be  dealt  with  by 
appropriate  measures.  Any  constitutional  cause,  such  as  gout,  must 
be  treated.  For  the  inflammation  of  the  nerve,  it  is  of  the  first 
importance  to  secure  to  the  part  as  perfect  rest  as  possible.  Move- 
ment causes  mechanical  irritation  of  the  nerve,  and  involves  functional 
stimulation  of  its  fibres,  both  of  which  are  injurious.  The  nerves  of 
a  limb  run  between  muscles,  and  the  contraction  of  these  muscles 
causes  pressui-e  on  the  inflamed  nerve,  and  irritates  it,  as  the  pain  thus 
produced  sufficiently  shows.  Pain  is  indeed  a  useful  indication  of  the 
harmful  influence  of  movement,  since  the  stimulation  of  the  nerves  of 
the  sheath,  in  which  the  pain  is  produced,  doubtless  has  also  a  reflex 
effect,  increasing  the  vaso- motor  element  in  the  infl "animation.  Hence 
it  is  most  important  that  all  movement  that  causes  pain  should  be 
avoided.  The  posture  of  the  limb  should  be  such  as  to  involve  the 
least  pain  and  the  least  danger  of  secondary  contracture.  The  general 
treatment  of  an  acute  neuritis  must  be  that  suitable  for  any  acute  local 
inflammation,  whatever  its  seat, — an  unstimulating  diet,  an  aperient, 
and  diuretics.  In  gouty  cases  a  brisk  purgative  may,  with  advantage, 
be  given.  General  diaphoresis  is  useful  in  cases  that  are  due  to  cold, 
and  in  others  local  sweating  is  often  of  distinct  service.  The  limb 
may  be  steamed,  or  exposed  to  hot  air.  This  should  be  followed  by 
hot  fomentations  applied  along  the  course  of  the  affected  nerve,  and 
these  by  linseed-meal  poultices.  Leeches  may  also  be  employed  at  the 
onset  of  severe  cases.  When  the  inflammation  results  from  injury, 
cold  may  be  applied  along  the  course  of  the  nerve  instead  of  heat; 
both  probably  modify  the  vascular  disturbance  of  inflammation  in  a 
similar  manner.  Counter-irritation  may  be  used  at  the  onset  of  slight 
cases ;  but  when  the  inflammation  is  severe,  this  agent  is  more  effective 
during  the  subsidence  of  the  inflammation  than  during  its  active 
stage.  Blisters,  repeated  mustard  plasters,  or  stimulating  liniments 
may  be  used,  but  care  must  be  taken  not  to  blister  skin  that  is 
ansesthetic,  or  troublesome  ulceration  may  be  caused.  The  same 
caution  is  necessary  in  regard  to  hot  applications,  as  is  shown  by  the 
case  mentioned  on  p.  79. 

Spontaneous  pain  requires  sedatives,  of  which  the  hypodermic  injec- 
tion of  morphia  is  by  far  the  most  potent.  It  should  be  used  only 
for  spontaneous  p;  in,  and  not  to  enable  the  patient  to  use  the  limb  in 
a  way  that  would  |  reduce  pain  if  morphia  were  not  given.     Mechanical 


TEEATMENT.  89 

irritation  may  be  equally  injurious,  although  the  pain  which  it  wotild 
cause  is  obviated  by  the  sedative.  Moreover  it  should  only  be  used 
as  a  last  resort,  when  the  agent  to  be  mentioned  next  is  inadequate. 
Morphia  has  little  influence  o^  the  morbid  process ;  it  lessens  the 
effect  of  the  disease  on  the  brain  only  by  acting  on  the  brain. 

In  cocaine  we  have  an  agent  capable  to  a  large  extent  of  replacing 
morphia,  and  of  a  greater  therapeutic  capacity.  Although  it  has  less 
power  of  relieving  pain,  it  is  capable  of  doing  much  more  than  that. 
It  arrests  the  local  transmission  of  the  impulses  that  cause  pain,  and 
so,  at  a  limited  region,  it  does  lessen  pain  and  often  remove  it  for  a 
time,  and  the  local  character  of  its  action  enables  it  to  exert  a  simul- 
taneous influence  on  the  morbid  process.  It  prevents  the  irritative 
influence  on  the  inflamed  structures  of  the  impressions  that  are  felt  as 
pain,  which  we  have  just  considered;  and  the  entire  arrest  of  the 
influence,  even  though  the  region  is  small,  seems  to  produce  a  much 
greater  effect  on  the  process  than  might  be  anticipated.  It  is  in- 
structive to  note  the  opposite  influence,  revealed  by  the  occasional 
redness  of  the  skia  and  subcutaneous  oedema,  along  the  course  of  the 
inflamed  nerve.  The  injection  should  be  made  at  one  of  the  seats  of 
pain.  In  the  rheumatic  form  salicylate  of  soda  has  been  given,  but  is 
of  doubtful  value.  Iodide  of  potassium  sometimes  seems  to  be  useful, 
but  no  agent  has  so  distinct  an  influence  on  the  process  of  inflam- 
mation in  the  nerve  as  small  doses  of  mercury.  A  grain  of  blue  pill 
may  be  given  once  or  twice  a  day ;  and  if  morphia  has  to  be  injected 
at  the  same  time,  the  mercury  is  useful  also  in  correcting  the 
constipating  influence  of  the  morphia. 

In  the  chronic  stage  or  form,  counter-irritation,  by  blisters  or 
cautery,  is  of  great  value.  So  also  is  electricity,  which  has  little 
influence  during  the  acute  stage.  The  voltaic  current  should  be 
used.  The  positive  electrode  may  be  placed  over  the  inflamed  part  of 
the  nerve,  or  over  the  seat  of  pain,  and  kept  there  for  ten  minutes  at 
a  time,  the  strength  of  current  being  slight,  such  as  the  patient 
can  just  perceive.  In  very  chronic  cases  a  stronger  current,  sufficient 
to  cause  actual  pain,  is  of  service,  applied  in  a  similar  n:anner,  but 
for  a  shorter  time.  It  has  probably  chiefly  a  counter-irritant  influence, 
and  faradism  may  be  used  in  the  same  way.  All  painful  impressions 
on  the  skin  lessen,  for  a  time,  the  nerve-pain. 

The  muscles  supplied  by  the  inflamed  nerve  may  be  left  alone, 
unless  their  wasting  is  marked,  or  the  degenerative  reaction  shows 
serious  damage  to  the  motor  fibres.  In  cases  of  moderate  severity 
they  will  recover  when  the  nerve  recovers.  All  that  is  desirable  is 
that  they  should  be  gently  rubbed  once  or  twice  a  day.  If  the  wasting 
is  considerable,  however,  or  there  is  degenerative  reaction,  they  should 
be  stimulated  to  gentle  contra  jcion  by  a  weak,  slowly  interrupted 
voltaic  current.  They  should  on  no  account  be  faradised  during  the 
active  stage  of  the  affection,  even  if  they  act  to  faradism.  The  acute 
pain    that    the   faradic   stimulus  causes,  and  the   increased  tender- 


30  NEUKOMA. 

ness  tliat  lasts  for  hours  afterwards,  are  evidence  of  its  injurioua 
effect. 

In  all  cases  attention  to  the  general  health  is  of  great  importance. 
Tonics  are  needed  during  the  chronic  stage.  Change  of  air  will  some- 
times remove,  in  a  few  weeks,  symptoms  that  have  previously  been 
stationary  for  months. 

The  modifications  of  treatment  that  are  rendered  necessary  by 
the  position  of  the  neuritis  will  be  considered  when  we  come  to  speak 
of  the  affections  of  the  several  nerves.  The  pathological  varieties  of 
neuritis  only  need  special  treatment  in  so  far  as  they  depend  upon 
special  causes,  and  this  point,  as  already  mentioned,  must  always  be 
one  of  the  first  considerations. 


MORBID  GROWTHS  IN  NERVES  :  NEUROMA. 

The  term  "neuroma"  has  been  applied  indiscriminately  to  all 
morbid  growths  situated  on  the  peripheral  nerves.  The  discoveiy,  by 
Virchow,  that  many  of  these  consist  of  a  growth  of  nerve-fibres,  while 
others  consist  of  heterologous  tissue  such  as  constitutes  morbid 
growths  elsewhere,  has  led  to  the  distinction  of  the  former  as  "  true 
neuromata  "  and  the  latter  as  "  false  neuromata,"  or  "  pseudo-neuro- 
mata," and  even  to  the  limitation  of  the  term  " neuroma"  to  a  growth 
of  nerve-tissue,  whether  in  the  central  or  peripheral  nervous  system, 
the  heterologous  growths  being  called  by  the  names  they  bear  in 
other  situations,  as  "  fibroma,"  "  sarcoma,"  &c.  The  latter  system  of 
nomenclature,  although  certainly  more  consistent,  has  not  become 
current  in  this  country. 

The  true  neuromata  may  consist  of  meduUated  or  of  non-medullated 
nerve-fibres,  termed  "  myelinic  "  and  "  amyelinic  "  forms  by  Yirchow. 
The  latter  were  for  a  long  time  regarded  as  fibrous.  The  occurrence 
of  ganglion-cells  has  been  proved  in  only  one  or  two  instances.  There 
is  connective  tissue  between  the  nerve-fibres,  which  varies  in  amount 
and  in  character,  and  hence  the  firmness  of  these  tumours  also  varies. 
This  interstitial  tissue  may  be  so  abundant  as  to  constitute  an  inter- 
mediate form  between  the  true  and  false  varieties.  This  is  probably 
the  condition  in  most  cases  of  multiple  neuromata.  The  "  false  neui-o- 
mata "  may  be  of  various  nature,  but  fibrous  growths,  "  fibromata," 
are  far  more  common  than  any  other  kind.  Myxoma  occasionally 
occurs,  the  new  mucoid  tissue  growing  from  the  nerve-sheath.  In 
one  case  *  a  myxo-fibroma  as  large  as   a  goose  egg  involved  the 

•  Schuster,  •  Zeitschr.  f.  Heilknnde,'  1886,  vii. 


NEUROMA. 


91 


slieatli,  and  pressed  on  the  median  nerve,  a  piece  of  which,  -when 
resected  with  the  tumour,  showed  hyaline  degeneration  of  the  medul- 
lary sheath,  the  axis-cylinders  persisting  in  a  slightly  swollen  condi- 
tion. Glioma  is  very  rare  on  the  peripheral  nerves,  although  occa- 
sionally found  on  the  auditory  nerve ;  it  should  be  remembered  that 
this  form  of  growth  arises  from  the  same  embryonal  tissue  as  true 
neuroma.  Various  forms  of  sarcoma  have  been  met  with.  Carci- 
noma also  occurs,  very  rarely  as  an  isolated  growth,  but  not  un- 
commonly as  a  more  or  less  diffused  or  nodular  infiltration  of  the 
nerve,  arising  by  extension  from  a  contiguous  growth.*  Syphilitic 
growths  are  common  on  the  cranial  nerves  within  the  skull,  but  are 
rare  elsewhere.  In  lepra  ansesthetica  the  nerves  are  infiltrated  with 
fibrous  tissue,  enlarging  them  to  many  times  their  normal  size.  The 
enlargement  is  rarely  nodular,  and  is  rather  a  chronic  cirrhotic 
inflammation  than  a  growth.     (See  Leprous  Neuritis.) 

A  curious  variety  of  neuroma,  consisting  of  interlacing  cords,  more 
or  less  nodular  and  tortuous,  is  termed  plexiform  neuroma.  The 
disease  commonly  begins  in  foetal  life,  and  is  most  common  on  some 
branch  of  the  fifth  nerve  in  the  orbit  or  the  upper  eyelid,  but  has  been 
met  with  over  the  temporal  bone,  in  the  lumbar,  cervical,  brachial, 
and  solar  plexuses,  on  the  penis  and  the  mamma.  It  may  be  quite 
superficial  or  deeply 
seated.  The  cords, 
from  1  mm.  to  seve- 
ral centimetres  in 
diameter,  consist  of 
a  clear  outer  zone  of 
concentric  fibrillar 
connective  tissue,  of 
inner  looser  nucle- 
ated tissue,  and,  in 
the  middle,  a  bundle 
of  nerve-fibres,  some 
normal,  others  de- 
generating. The 
cords  are  connected 
together  by  loose  tissue,  sometimes  myxomatous,  separated  from 
the  proper  tissue  of  the  cords  by  an  epitheliated  space.  The  growth 
of  this  form  is  extremely  slow,  but  it  may  exert  compression  on 
adjacent  structui'es.f  A  remarkable  case  of  extensive  myxomatous 
disease  of  the  nerves  of  the  forearm,  congenital  in  origin,  pre- 
senting analogies  to  plexiform  neuroma,  is  recorded  by  Mr.  De 
Morgan.  J 

*  A  unique  case  of  rliiibdomyoma  occurring  jr-   a  nerve  is  recorded  by  Orlandi 
('Arch.  p.  1.  sc.  rned.,'  xix,  5). 

t  Maichand,  '  Viichow's  Archiv,'  Bd.  Ixxvi,  p.  36. 
J  '  Path.  Trans.,'  vol.  xxvi,  p.  2. 


Fia.45. — Plexiform nenioma from theorbit.  (After Mar- 
chand.)  The  connective  tissue  surrounding  tlie  corda 
has  been  removed  except  at  «,and  irregular,  cylindrital, 
nodular  cords  are  seen  anastomosing.  A  nerve  pussea 
into  the  tumour  and  suddenly  becomes  enlarged. 


'%d^ 


92  NEUROMA. 

The  subcutaneous  extremities  of  sensory  nerves  are  sometimes 
enlarged  into  minute  tumours  which,  when  painful,  have  been  termed 

"  tubercula  dolorosa."  Neuromata  in 
the  skin  may  co-exist  with  similar 
tumours  on  the  nerve-trunks.  Those 
shown  in  Pig.  46  are  from  the  same 
case  as  the  tumoui's  represented  in 
.1  I    Figs.  47—49. 

The   size   attained  by  growths   on 
nerves  varies  from  that  of  a  child's 
head   to   a  nodule   only  just  visible. 
They  rarely  exceed  the  dimensions  of 
Pia.  46. -Cutaneous  neuromata  from  the  closed  fist.     The  variation  in  size 
skin  of  abdomen.     (After  Smith.)        »         i.  •   i  j      •    -n      ,      .    -i  . 

'    01  multiple  neuromata  is  illustrated  in 

Figs.  47 — 49,  after  Smith.*     The  large  tumour  (Pig.  47)  was  the  size 
of  a  lemon,  but  in  the  same  case  a  growth  from  the  sciatic  plexus 

Fio,  47.  Fig.  48.  Fia.  49.  Fi&.  50. 


\J  u 


FiG.  47. — Right  elbow  of  a  man,  set.  30,  with  multiple  neuromata;  those  on  the 
nerves  of  the  arm  are  visible  beneath  the  skin  as  moniliform  series  of  swellings 
along  the  course  of  the  nerves-     (After  Smith.) 

Fig.  48. — Part  of  the  brachial  plexus  and  nerves  of  the  arm. 

Fig.  49. — Neuroma  of  the  posterior  tibial  nerve,  same  case.  The  tumour  was  the 
size  of  a  lemon,  and  could  be  felt  in  the  popliteal  space.  The  flattened  bundles  of 
fibres  of  the  nerve  were  separated  and  passed  over  the  surface  of  the  tumour.  The 
portion  of  nerve  to  the  right  is  a  piece  ot  the  external  popliteal.  The  tumours  caused 
no  interference  with  function.  The  patient  died  of  enteric  fever.  One  large  tumour 
tilled  the  pelvis. 

Fig.  50. —  Small  neuroma  of  popliteal  nerve  laid  open.  It  is  seen  to  lie  within  the 
sheath.     It  caused  no  symptoms  during  life.     (After  Smith.) 

almost  filled  the  pelvis.  They  may  occur  on  any  nerve  in  the  body, 
cranial  or  spinal,  and  are  sometimes  numerous  on  the  pneumogastrics, 
spinal  roots,  and  sympathetic  plexuses.    When  neuromata  are  multiple 

*  R.  W.  Smith,  '  Treatise  on  Neuroma,'  1849.  Many  instructive  cases  have  been 
collected  by  Bowlby  (•  Diseases  and  Injuries  of  Nerves'). 


CAUSES.  93 

they  are  often  extremely  numerous,  and  almost  every  nerve  in  the 
body  may  be  transformed  into  a  chain  of  growths.  In  the  case 
figured  more  than  200  were  counted  in  the  right  arm  alone,  and  the 
total  number  of  growths  in  the  body  cannot  have  been  less  than  1000, 
while  in  another  3020  were  counted.*  These  multiple  neuro-fibromata 
have  a  great  tendency  to  undergo  sarcomatous  degeneration.  They 
have  hence  been  termed  "  secondarily  malignant  neuromata. "f 

The  tumours  are  almost  always  within  the  sheath  of  the  nerve 
(Pig.  50).  Sometimes  they  are  on  one  side,  and  the  nerve  may  pass 
by  unchanged.  More  often  the  substance  of  the  nerve  is  involved, 
and  the  fibres  may  be  separated  and  spread  out  on  the  surface 
(Fig.  49).  Even  then  they  may  not  be  damaged.  They  suffer  far 
more  in  heterologous  growths  than  in  true  neuromata. 

Causes. — The  causes  of  neuroma  are  generally  obscure.  Multiple 
neuromata  are  sometiines  hereditary,  and  are  probably  due,  in  most 
cases,  to  a  congenital  tendency  of  tissue  growth.  Plexiform  and 
multiple  neuromata  have  been  met  with  in  the  same  family.  They 
are  said  to  be  sometimes  the  result  of  general  neurotic  predisposition, 
and  evidence  of  this  is  found  in  the  occasional  occurrence  of  neuro- 
mata in  the  subjects  of  cretinism  or  idiocy,  of  which  some  remarkable 
examples  have  been  recorded.  It  is  now  generally  held  that  multiple 
and  plexiform  neuromata,  together  with  the  affection  kaown  as  ele- 
phantiasis neuromatodes,  are  results  of  the  same  congenital  process.  J 
Multiple  neuromata  are  said  to  be  almost  confined  to  men.  The 
isolated  form  is  not  uncommon  in  women.  They  may  occur  at  any 
age.  Yirchow  believes  that  they  are  unusually  common  in  the 
phthisical  and  scrofulous. 

Of  traceable  immediate  causes  the  most  frequent  is  traumatic 
injury.  Pressure,  punctured  wounds,  or  division  of  the  nerves  may 
give  rise  to  them,  apparently  by  a  perversion  in  the  process  by  which 
cicatrisation  takes  place.  A  well-known  instance  is  the  formation  of 
the  so-called  "  amputation  neuromata,"  or  "  bulbous  nerves,"  round 
or  oval  growths,  the  size  of  a  bean  or  larger,  which  form  on  the 
divided  extremities  of  the  nerves  in  the  stump  left  after  an  amputation. 

The  occurrence  of  new  growths  containing  nerve-fibres  may  seem  to 
be  very  remarkable.  We  must  remember,  however,  how  frequently 
they  are  connected  with  traumatic  causes,  i.  e.  take  origin  in  cicatricial 
processes.  In  such  processes  the  nerve-fibres  present  a  remarkable 
power  of  growth.  Ranvier  has  shown  that  from  the  end  of  each  old 
fibre  several  new  fibres  grow,  only  one  of  which  probably  ultimately 
persists.  Under  apparently  mechanical  influences  some  of  these  fibres 
may  twist  about,  and  even  turn  and  grow  upwards.     It  is  thus  not 

•  'Med.  Times  and  Gaz.,'  1883,  i,  p.  152. 

f  Garre,  '  Beitrage  z.  klin.  Cliir.,'  ix ;  Sclieven,  ibid.,  xvii. 

J  See  especially  Finotti,  '  Virch.  Arch.,*  cxliii.  Some  go  so  far  as  to  include 
molluscam  fibrosnm  in  the  same  category  (Soldau,  '  Inaug.  Diss.,'  Berlin,  quoted  in 
•Virch.  Jahrb.,'  xxx,  i,  p.  231). 


94  NEUilOMA. 

diflBcult  to  understand  ttat  a  true  neuroma  may  result  from  injury  tc 
a  nerve ;  it  is  said,  indeed,  that  the  ends  of  the  nerves  in  amputation 
stumps  are  generally  enlarged  into  minute  bulbs,  which  contain  more 
fibres  than  do  the  nerves  on  which  they  are  situated.* 

Symptoms. — These  may  be  entirely  absent,  especially  in  the  case  of 
multiple  (true)  neuromata,  which  are  sometimes  discovered  post 
mortem  when  their  existence  has  not  been  suspected.  Often,  how- 
ever, they  cause  marked  symptoms.  The  most  frequent  is  pain 
seated  in  the  tumour  (which  may  be  extremely  tender)  or  referred  to 
the  distribution  of  the  nerve,  often  acute,  burning,  or  darting,  and 
frequently  intermittent.  It  may  be  increased  by  pressure  on  the 
tumour.  In  the  case  of  the  terminal  neuromata  local  pain  may  be  the 
only  sensory  symptom ;  but  when  the  nerve-fibres  suffer,  parsesthesise, 
numbness,  or  formication  may  be  complained  of,  and  ultimately  sen- 
sation may  be  lessened  or  lost.  Paralysis  of  the  muscles  supplied  by 
the  nerve  is  occasionally  met  with,  the  region  paralysed  corresponding 
to  the  distribution  of  the  nerve  on  which  the  tumour  is  situated, 
except  in  the  case  of  neuromata  in  the  cauda  equina,  from  which 
paraplegia  may  result,  with  flexor  spasm.  A  neuroma  of  the  pneumo- 
gastric  may  cause  grave  cardiac  disturbance,  sometimes  increased  by 
pressTire  on  the  tumour. 

More  common  motor  symptoms  are  reflex  spasms  in  adjacent  or 
even  distant  muscles.  For  instance,  in  a  case  of  "  bulbous  nerves  " 
in  the  stump  after  amputation  at  the  shoulder- joint,  the  muscles  on 
that  side  of  the  neck  were  in  constant  clonic  spasm.  Occasionally 
epileptiform  convulsions  are  produced,  and  have  been  known  to  cease 
when  a  painful  neuroma  had  been  excised.  The  occurrence  of  these 
symptoms  has  little  relation  to  the  size  of  the  tumour. 

When  the  neuromata  are  in  accessible  situations  they  may  be  felt. 
Sometimes  they  give  rise  to  visible  tumours,  and  in  multiple  neuroma 
the  course  of  the  superficial  nerves  may  be  marked  out  by  lines  of 
bead-like  swellings,  as  in  Fig.  47.  The  cutaneous  neuromata  also 
cause  visible  swellings.  The  whole  abdomen  was  covered  with  such 
nodules  in  the  case  recorded  by  Smith  (Fig.  46).  They  are  sometimes 
not  sensitive,  sometimes  exquisitely  tender  and  most  painful,  especially 
when  closely  united  with  the  skin.  Pressure  on  the  nerve  above  the 
tumour  commonly  lessens  the  pain. 

Plexiform  neuroma  rarely  causes  other  symptoms  than  the  signs 
manifesting  its  local  presence.  The  functions  of  the  nerve-fibres 
involved  are  not  usually  interfered  with.  Once  or  twice  anaesthesia 
of  the  skin  has  been  observed. 

Course. — The  growth  of  neuromata  varies  greatly  in  rapidity. 
When  symptoms  occur  they  most  commonly  last  for  a  long  time, 
increasing  in  severity,  and  f  vom  the  long-continued  pain  the  patient 
may  ultimately  be  worn  out.     In  other  cases  the  symptoms  show 

•  Bowlby,  loc.  cit.,  p.  20. 


DIAGNOSIS — PROGNOSIS TEEATMKNT.  95 

little  tendency  to  increase,  and  in  rare  instances  they  may  lessen  in 
severity  and  even  disappear. 

Diagnosis. — The  recognition  of  the  existence  of  a  neuroma  depends 
on  its  superficial  position,  or  on  the  production  of  symptoms  of  damage 
to  the  nerve.  The  latter,  when  existing  alone,  are  equivocal,  since  they 
may  be  due  to  pressure  on  the  nerve  from  an  adjacent  growth,  or  even 
to  neuritis.  The  disease  may  be  suspected  if  symptoms — pain,  numb- 
ness, weakness — occur,  limited  to  the  distribution  of  a  nerve-trunk, 
and  no  cause  of  external  pressure  can  be  discovered,  and  the  long 
course  of  the  symptoms  makes  it  unlikely  that  they  are  due  to 
neui'itis.  The  diagnosis  can,  however,  only  be  certainly  made  when 
the  tumour  can  be  felt.  Secondary  subcutaneous  sarcomata  may  very 
closely  resemble  neuromata. 

The  diagnosis  of  the  kind  of  neuroma,  whether  "  true  "  or  "  false," 
i.  e.  composed  of  nerve-fibres  or  other  tissue,  is  very  difiicult.  Multiple 
neuromata  are  usually  true,  and  of  very  slow  growth ;  false  neuromata 
are  rarely  multiple  unless  they  are  of  infecting  nature  and  rapid  in 
development.  The  existence  of  idiocy,  or  other  neuropathic  indication, 
is  in  favour  of  the  neural  nature  of  the  tumours.  The  lateral  position 
of  the  growth  on  the  nerve  is  in  favour  of  its  "false  "  character. 
The  diagnosis  of  plexif  orm  neuroma  is  only  possible  when  the  tortuous 
nodular  cords  can  be  felt. 

Peognosis. — This  depends  on  the  existence  of  symptoms.  If  these 
are  absent,  unless  the  tumour  is  of  rapid  growth,  there  is  good  reason 
to  hope  that  they  will  not  occur.  In  multiple  (true)  neuromata  the 
nerve  is  less  likely  to  suffer  than  in  isolated  growths.  If  symptoms 
are  present  the  jDrognosis  is  less  favorable,  and  depends  on  the  ten- 
dency which  they  exhibit  to  increase. 

Treatment. — Medicines  are  useless  except  in  the  syphilitic  forms 
of  nerve-tumour,  not  here  considered.  Extirpation  is  the  only  remedy. 
If  the  tumour  is  lateral,  and  can  be  shelled  out,  the  removal  entails 
little  risk.  If,  however,  the  growth  infiltrates  the  substance  of  the 
nerve,  the  affected  part  must  be  excised,  and  the  ends  brought 
together.  The  risk  of  permanent  loss  of  function  in  the  nerve  is 
great,  and  in  deciding  on  such  an  operation,  the  urgency  of  the 
symptoms,  and  the  importance  of  the  function  of  the  nerve,  must  be 
taken  into  consideration.  After  the  extirpation  of  such  tumours,  the 
tendency  of  cicatricial  processes  in  nerves  to  go  on  to  the  formation 
of  similar  growths  (seen  in  "  bulbous  nerves  ")  renders  the  chance  of 
relapse  considerable.  The  operation  reproduces  conditions  favorable 
for  the  activity  of  whatever  predisposing  influence  aided  in  the  original 
development  of  the  disease.  When  neuromata  are  multiple,  surgical 
treatment  is  out  of  the  question,  excep"'.  for  isolated  tumours  that 
cause  distress. 

In  the  case  of  terminal  neuromata — whether  the  cutaneous  "  tuber- 
cula  dolorosa  "  or  the  amputation  neuromata — excision  is  practicable, 
and  it  us  unattended  with  the  risks  attending  interference  with  a 


96  ^  SPINAL    NERVES. 

nerve  in  its  continuity.  In  amputation  flaps  the  chance  of  a  relapse 
is  considerable. 

In  plexif  orm  neuroma  excision  is  undesirable,  and  no  other  treatment 
is  of  any  use. 

Pain,  and  the  reflex  spasm,  that  are  so  distressing  in  amputation 
neuromata,  need  relief  when  attempts  to  cure  have  failed.  By  nerve- 
stretching  we  may  perhaps  give  relief  without  the  effects  that  follow 
excision,  and  in  cocaine  we  have  a  means  of  arresting  for  a  time  the 
ingoing  impulses  that  play  an  important  part  in  generating  the 
symptoms.  The  rest  thus  given  to  the  centre  is  absolute,  and  its 
degree  and  frec^uent  repetition  may  compensate  for  its  brevity. 


DISEASES  OF  SPECIAL  NERVES. 

The  diseases  of  the  cranial  nerves  may  be  most  conveniently 
considered  in  connection  with  the  diseases  of  the  braia.  Her«, 
therefore,  only  the  affections  of  the  spinal  nerves  will  be  described, 
in  so  far  as  they  present  special  features.  The  effect  of  paralyais 
of  the  individual  muscles  has  been  already  described  in  detail,  and 
need  not  be  here  repeated,  except  in  general  terms,  or  in  so  far  as  the 
association  of  palsies  resulting  from  a  nerve-lesion  presents  a  parti- 
CTolar  character. 

Phebnio  Nerve. — Impaired  function  of  the  phrenic  nerve  is 
commonly  the  result  of  disease  of  the  spinal  cord,  or  of  the  roots  of 
the  nerve — the  third  and  fourth  cervical  trunks — from  disease  of  the 
membranes  or  the  bones.  The  deep  position  of  the  nerve-trunk  pro- 
tects it  from  injury,  but  it  is  occasionally  damaged  in  wounds  of  the 
neck,  and  in  its  course  through  the  thorax  it  may  be  compressed  by 
tumours,  aneurisms,  &c.,  or  involved  in  inflammations  of  the  pleura. 
Paralysis  of  one  nerve .  sometimes  follows  exposure  to  cold,  and  is 
ascribed  to  neuritis.  That  of  both  has  been  met  with  in  multiple 
neuritis ;  it  has  also  been  observed  following  diphtheria  and  influenza, 
in  the  course  of  tabes  (Grerhardt)  and  progressive  muscular  atrophy, 
and  in  poisoning  by  lead  and  alcohol.  In  disease  of  the  spinal  cord 
and  membranes  both  nerves  are  usually  paralysed ;  causes  acting  on 
the  nerve-trunk  usually  affect  one  only.  The  effect  and  symptom  of 
paralysis  is  inaction  of  the  diaphragm  (see  p.  33).  If  one  nerve  only 
is  affected,  the  diaphragm  does  not  descend  on  that  side,  but  the 
movement  of  the  other  side  lessens  the  resulting  defect  of  movement, 
and  it  can  then  be  detected  only  by  close  observation.  The  loss  of  the 
action  of  the  diaphragm  has  little  effect  on  the  respiratory  functions 
while  the  patient  is  at  rest,  but  dyspnoea  is  said  to  be  readily  pro- 
duced by  exertion ;  the  breathing  then  becomes  quick  and  the  voice 


NERVES    OP   THE    ARM.  97 

feeble.  At  the  same  time  this  is  probably  chiefly  because  too  much 
and  too  constant  work  is  thrown  on  the  upper  thoracic  muscles  of 
extraordinary  breathing.  Any  lung  disease,  such  as  an  attack  of 
bronchitis,  is  rendered  far  more  serious  by  the  diminished  breathing 
power.  When  the  diaphragm  is  paralysed,  the  movement  of  the 
thorax  is  often  increased,  and  the  expansion  of  the  lower  part  may 
draw  forwards  the  adjacent  abdominal  wall.  This  must  not  be  mis- 
taken for  the  effect  of  the  descent  of  the  diaphragm.  Paralysis  has 
to  be  distinguished  from  (1)  abnormal  ner\  o  is  breathing.  The  dia- 
phragm is  used  little  in  extraordinary  breathing,  which  is  chiefly  by 
the  upper  part  of  the  thorax,  of  the  "  superior  costal  type,"  as  it  is 
called.  Hysterical  and  nervous  patients  will  often  breathe,  for  a 
time,  only  in  this  manner,  even  when  at  perfect  rest,  especially  when 
they  are  under  observation.  Such  breathing  is  no  doubt  faciUtated 
in  women  by  the  fact  that  the  diaphragm  is  habitually  used  by  them 
less  than  by  men.  Repeated  examination  may  be  necessary  to  deter- 
mine whether  there  is  any  real  paralysis  of  the  diaphragm  in  these 
cases.  A  single  diaphragmatic  inspiration  settles  the  point.  The 
patient's  attention  should  be  distracted,  and  she  should  not  be  aware 
of  the  object  of  the  examination.  (2)  Inflammation  of  the  diaphragm 
may  arrest  its  movement,  and  so  also  may  diaphragmatic  pleurisy  or 
peritonitis — each  distinguished  by  the  fact  that  it  is  usually  secondary 
to  adjacent  inflammation,  and  any  movement  causes  characteristic 
pain.  (3)  A  primary  and  isolated  degeneration  of  the  muscular  fibres 
of  the  diajohragm  has  been  described  by  Callender  and  others  as  com- 
mon after  death,  but  it  has  not  yet  been  proved  to  cause  symptoms, 
and  needs  further  investigation. 

Paralysis  may  be  due  to  disease  of  the  nerve,  its  roots,  or  of  the 
spinal  cord.  In  the  latter  case  other  muscles  always  suffer ;  in  the 
former  the  diaphragm  usually  suffers  alone,  the  result  of  peri- 
pheral neuritis.  The  distribution  and  the  course  of  the  other  palsies 
are  distinctive.  By  far  the  most  common  cause  is  disease  of  the  spinal 
cord,  local  or  ascending  from  below  and  adding  this  final  effect  to  the 
preceding  palsy  of  the  intercostals.  In  disease  of  the  nerve-roots  there 
are  other  indications  of  the  position  of  the  disease.  The  affection  is 
often  overlooked  because  not  searched  for,  especially  in  local  disease 
of  the  cervical  cord.  The  observer  forgets  that  its  associations  are  not 
with  the  lower  dorsal  muscles,  and  he  omits  to  look  for  it  in  cases  in 
which  the  cervical  cord  is  suffering  and  other  respiratory  muscles  act 
weU. 

If  there  is  reason  to  suspect  partial  neuritis  as  from  cold,  hot 
fomentations  at  first,  and  afterwards  cotmter-irritation,  should  be 
applied  over  the  lower  and  inner  part  of  the  anterior  triangle  of  the 
neck.  The  only  other  special  point  in  treatment  is  the  appHcation  of 
electricity.  The  nerve  may  be  stimulated  by  pressing  the  rheophore 
deeply  outside  the  lower  part  of  the  clavicular  portion  of  the  sterno- 
mastoid.     The  other  pole  may  be  placed  at  the  epigastrium  or  over  the 

VOL.  I.  7 


9o  SPINAL  NERVES. 

corresponding  lialf  of  the  diaphragm.  But  the  influence  of  electricity 
on  paralysis  of  the  diaphragm  is  not  sufficient  to  make  its  use  desir- 
able. In  the  cases  in  which  such  treatment  could  do  good  the  affec- 
tion is  transient  and  comparatively  unimportant.  In  central  disease 
electricity  has  very  little  influence. 


Neetes  of  the  ITppeb  Limb. 

The  nerves  of  the  arm  and  shoulder  are  derived  from  the  five  lovrer 
cervical  and  the  first  dorsal  nerves.  These  interlace  in  the  brachial 
plexus  in  such  a  complex  manner  that  most  of  the  nerves  of  the  arm 
are  derived  from  many  spinal  roots. 

The  nerve-roots  form,  by  their  union,  three  trunks,  which  we  raay  indicate  by 
Roman  numerals.  They  are  formed  thus  : — I,  by  the  branch  from  the  fourth,  and 
the  roots  of  the  fifth  and  sixth  cervical ;  II,  by  the  seventh ;  and  III,  by  the  eighth 
cervical  and  the  first  dorsal.  Each  trunk  divides  into  two  parts,  and  the  union  of 
these  divisions  forms  the  three  cords  of  the  brachial  plexus  from  which  the  nerves 
of  the  arm  proceed.  But  before  the  primary  trunks  divide,  certain  nerves  arise, 
the  origin  of  which  is  thus  less  doubtful.  The  fifth  and  sixth  cervical  roots  give 
origin  directly  to  the  posterior  thoracic  nerve  for  the  serratus,  and  from  the  cord 
formed  by  their  union  springs  the  supra-scapular  nerve.  The  three  cords  of  the 
plexus  have  the  following  relations: — The  posterior  is  derived  from  all  three 
primary  trunks,  and  gives  rise  to  the  subscapular  nerve,  the  cireninflex,  and  the 
musculo-spiral  (or  radial,  as  the  whole  nerve  is  sometimes  termed).  The  upper 
or  outer  cord  is  derived  from  the  two  upper  primary  trunks,  i.  e.  from  the  fourth, 
fifth,  sixth,  and  seventh  cervical  roots,  and  from  it  proceed  one  anterior  thoracic 
and  the  musculo-cutaneous  nerves,  together  with  the  outer  head  of  the  median. 
The  inner  or  lower  cord  is  derived  only  from  the  lowest  primary  trunk,  '.  . 
from  the  last  cervical  and  first  dorsal,  and  gives  rise  to  the  ulnar,  the  inner 
head  of  the  median,  the  internal  cutaneous,  the  intercosto-humeral,  and  to  the 
second  anterior  thoracic  nerves.  It  may  be  convenient  to  put  these  coarse 
anatomical  relations  in  the  form  of  a  table. 


Nerves. 


Primary  Trunks, 


Nerves, 


Subscapular    ••«    _ 
Circumflex       .1  ' 

Musculo-spiral  J 


Upper 
cord 


Lower 
cord 


fExtemal  ant.  thoracic. 
J  Musculo-cutaneous. 
[Outer  head  of 'j 

j-  median. 
/•Inner  head  of  J 
Ulnar. 

Internal  cutaneous. 
Intei-costo-humeral. 
^Internal  ant.  thoracic. 

These  anatomical  facts,  however,  give  us  little  help  in  tracing  the  relation  of 
the  nerves  to  the  spinal  roots.      The  investigations  of  Ferrier  and  Yeo,*  who 

*  'Pioc.  Roy.  Soc.,'  March  2L>t,  1881.  Careful  dissections  by  Herringliara  (cf. 
March  25th,  1887)  give  somewhat  different  results,  but  this  method  of  investigation 
is  open  to  more  uncertainties  than  that  of  stimulation.  Herringham's  conclusiona 
.should,  however,  be  noted  by  future  investigators. 


NEEVES    OP    THE    AEM.  99 

ascertained  tlie  movements  produced  by  faradisingthe  several  spinal  roots  in  the 
monkey,  show  us  the  way  in  which  the  movements,  muscles,  and  nerves  are 
represented  in  the  spinal  roots.  Their  results  are  therefore  of  great  interest, 
although  we  are  not  justified  in  transferring  the  facts  to  man  except  in  bo  far 
as  they  receive  confirmation  from  human  anatomy  and  pathology.*  Subject  to 
this  reservation,  the  chief  results  are  important,  and  may  be  thus  stated  rf 

The  roots  to  which  the  several  nerves  are  thus  traced  are  as  follows : — Sub- 
Bcapular,  6  and  7  C. ;  circumflex,  4  and  5  C. ;  musculo-spiral,  4,  5,  6,  7,  8  C. ; 
musculo-cutaneous,  4,  5  C. ;  median,  5,  6,  7,  8  C. ;  ulnar,  8  C,  1  D. 

Still  more  important  are  their  observations  on  the  relation  of  various  move- 
ments of  the  arm  to  the  nerve-roots,  and  these  are  as  follows: 

Cervical  4i.—  Elevation  and  retraction  of  the  arm,  flexion  and  supination  of 
the  forearm;  by  the  rhomboids,  supra-  and  infra-spinatus,  biceps,  brachialis, 
and  supinators. 

Cervical  6.— Similar  to  the  last,  but  without  retraction  of  the  arm,  and  with 
extension  of  the  wrist  and  first  phalanges ;  by  the  deltoid,  serratus,  flexors  of 
elbow,  extensors  of  wrist,  and  long  extensors  of  fingers. 

Cervical  6. — Adduction  and  retraction  of  the  upper  ai-m,  extension  and  pro- 
nation of  the  forearm,  flexion  of  the  wrist ;  by  contraction  of  the  pectoralis, 
latissimus  dorsi,  triceps,  flexors  of  wrist,  pronators. 

Cervical  7. — Adduction  and  rotation  inwards  at  the  shoulder-joint,  flexion  of 
the  wrist,  and  of  the  fingers  at  the  second  phalanx;  by  the  teres  major, 
latissimus  dorsi,  subscapularis,  triceps,  and  long  flexors  of  the  fingers. 

Cervical  8. — Flexion  of  fingers  and  thumb  so  as  to  close  the  fist ;  flexion  of 
wrist  towards  the  ulnar  side,  pronation  of  forearm,  extension  of  elbow ;  by  the 
intrinsic  muscles  of  the  hand,  the  long  flexors  of  the  fingers  and  thumb,  the 
flexors  of  the  wi-ist  and  the  triceps. 

Dorsal  1. — Adduction  of  the  thumb,  flexion  of  the  fingers  at  the  metacarpo- 
phalangeal joints  ;  by  the  interossei,  &c. 

Thus  most  movements  are  related  to  many  spinal  roots.  The  nerves  supplying 
each  muscle  have  a  correspondingly  multiple  relation  to  the  spinal  roots.  It 
cannot  be  traced  anatomically,  but  has  been  ascertained  by  comparing  the 
results  of  experiments  on  animals  with  those  of  disease  and  injury  in  man,  and 
(in  the  case  of  sensation)  of  the  division  of  roots  for  the  treatment  of  some 
forms  of  disease.  The  facts  thus  ascertained  have  been  confirmed  by  cases  of 
disease  of  the  spinal  cord  itself,  which,  when  sufficiently  limited  to  be  significant, 
have  been  found  to  agree  closely  with  observations  on  the  roots.  The  con- 
stituents of  each  nerve-root  come  from  a  definite  part  of  the  cord,  which  is 
termed  the  corresponding  spinal  "segment."  A  single  muscle  is  seldom 
related  to  a  single  segment ;  usually  its  nerve-fibres  come  from  pai-ts  of  two 
segments,  or  even  from  three.  Its  representation  in  the  grey  matter  of  the  cord 
corresponds  to  its  relation  to  the  nerve-roots,  and  has  often  a  considerable 
vertical  extent.  The  most  important  relations  are  these  :  that  of  the  deltoid, 
rhomboids,  supra-  and  infra-spinatus,  flexors  of  the  elbow,  and  supinators  to 

*  In  the  case  of  the  leg  there  are  some  important  discrepancies  between  the 
results  in  the  ape  and  the  conditions  that  obtain  in  man.  These  will  be  noticed  in 
their  proper  place. 

t  Ferrier  has  since  stated  that  the  relations  he  gave  (followed  in  the  text)  were  all 
one  nerve  too  high  ('  Proc.  Roy.  Soc.,'  1883,  vol.  xxxv,  p.  229),  but  this  would  make 
the  itmervation  of  the  intrinsic  muscles  of  the  hand  from  the  second  dorsal  uerve^ 
which  is  certainly  not  the  case  in  man. 


100 


SPINAL   NERVES, 


the  fourth  and  fifth  cervical ;  that  of  the  adductors  of  the  arm  and  extensort 
of  the  elbow  to  the  sixth  and  seventh  nerves;  pronation  to  the  sixth  and 
eighth ;  extension  of  the  wrist  to  the  fifth  ;  flexion  to  the  eighth  ;  extension  of 
the  first  phalanx  to  the  fifth;  flexion  of  the  fingers  to  the  seventh  and  eighth, 
and  the  action  of  the  intrinsic  muscles  of  the  hand  to  the  first  dorsal.  These 
facts  will  probahlj,  in  the  future,  find  important  practical  applications. 

Eegarding  the  relation  of  the  sensory  branches  to  the  nerve-roots,  we  have, 
hesides  the  facts  of  anatomy,  only  the  evidence  furnished  by  rare  cases  of 
injury  or  disease  in  which  the  lesion  and  its  effects  are  well  defined.*  Few 
facts  of  this  character  are,  however,  so  conclusive  as  that  illustrated  in  Fig.  51,  in 
which  the  area  shaded  by  slanting  lines  was  rendered  anaesthetic  by  the  division 
of  the  posterior  roots  of  the  last  cervical  and  first  dorsal  nerves.  The  general 
indications  are  that  the  distribution  of  the  sensory  fibres  of  the  nerve-roots  is 
along  the  axis  of  the  limb,  and  not  across  it.  The  last  two  pairs  of  nerves 
supply  the  ulnar  side  of  the  limb  and  the  finger  tips,  the  fifth  pair  the  radial 
side  and  the  middle  roots  of  the  brachial  plexus,  the  middle  zone  of  each  side  of 
the  limb  and  the  thumb.f 


ilUlUi^ 


^'^  '  ^jijjjJ^^'mim^^ 


Fig.  51. — Area  of  impaired  sensation  after  division  of  the  posterior 
branches  of  tlie  8th  C.  and  1  D.  nerves,  by  Professor  Horsley  for  inveterate 
paroxysmal  neuralgia  in  that  region. 


Paralysis  of  tlie  nerves  of  the  upper  limb  may  be  due  to  disease  of 
tbe  nerve-roots,  of  the  plexus,  or  of  the  trunks  that  arise  from  these. 
It  is  convenient  to  consider  first  the  diseases  of  the  nerve-trunks,  and 
afterwards  those  of  the  nerve-roots  and  of  the  plexus.  From  the 
roots  themselves  only  one  important  nerve-trunk  is  derived : — the  pos- 
terior thoracic  nerve.     The  others  spring  from  the  plexus. 

'±he  morbid  influences  that  affect  the  nerves  of  the  arm  are  veiy 
varied.  The  brachial  plexus  passes  close  to  the  shoulder- joint,  and 
hence  dislocation  often  damages  the  nerves,  sometimes  one  only,  some- 
times several,  and  occasionally  all  the  trunks.     In  fractures  of  the 

*  Instructive  facts  of  this  character  will  be  found  in  papers  by  Thorburn, 
*  Brain,'  January,  1887,  and  October,  1887,  Allen  Starr  ('American  Journal  of  the 
Medii-al  Sciences/  1892),  and  Head  ('  Brain,'  1893  and  1894),  who  has  mapped  out 
tlie  areas  representing  the  segmental  distribution  of  the  sensory  fibres  of  the  spinal 
ci'rd  by  means  of  obsei-vations  on  superficial  tenderness  accompanying  visceral 
disease,  on  cases  of  herpes  zoster,  and  on  cases  of  spinal  cord  disease.  (See  diagrams 
at  end  of  Chapter  on  "  Functions.") 

t  This  is  due  to  the  mode  of  growth  of  the  arm-bud  in  the  embiyo,  as 
shown  by  Ross  in  a  most  instructive  paper,  '  Brain,'  January  7th,  1888.  See 
also  chapter  on  ilie  Functions  of  the  Spinal  Cord. 


NERVES    OF    THE    ARM.  101 

bones  the  nerves  adjacent  may  be  torn,  or  may  be  compressed  by 
the  ends  of  the  bone,  or  by  the  callus  that  is  formed,  especially  by  the 
exterior  "  fibrous  callus.'*  The  musculo-spiral  nerve  is  most  prone  to 
suffer  from  its  proximity  to  the  humerus,  and  in  one  such  case  the 
nerve,  when  exposed,-  was  found  not  only  to  be  embedded  in  the  callus 
tissue,  hut  to  have  been  divided  for  about  one-third  of  its  diameter, 
opposite  the  fracture.  Bandages  that  are  applied  are  occasional  sources 
of  compression,  and  there  is  especial  danger  of  harm  from  those  that 
secure  a  splint  applied  to  prevent  motion  in  cases  of  neuritis.  Unless 
loss  of  sensation  draws  attention  to  it,  the  injury  is  generally  only  dis- 
covered when  the  splint  is  given  up,  and  therefore  its  possibility  should 
be  remembered.  The  course  of  certain  nerves  is,  in  some  parts  of 
the  arm,  especially  apt  to  be  directly  involved  in  wounds  of  various 
kinds,  or  they  may  be  compressed  by  the  cicatricial  fibrous  tissue  when 
the  wound  is  merely  near  the  nerve. 

The  position  of  some  exposes  them  to  pressure  against  the  unyielding 
bone.  .Even  slight  pressure  on  these  nerves  is  effective  when  prolonged, 
as  it  often  is  during  sleep,  when  warning  sensations  are  unperceived. 
The  sleep  induced  by  alcohol  is  especially  apt  to  be  attended  by  this 
accident.  Hence  there  is  a  group  of  "  8leep-palsies "  of  the  arm, 
chiefly  of  the  muscido- spiral  and  median  nerves,  rarely  of  the  plexus 
itself.  One  nerve,  the  musculo-spiral,  passing  between  the  bone  and 
the  triceps,  is  occasionally  bruised  by  the  contraction  of  the  muscle. 

Primary  neuritis  may  involve  any  nerve,  or  the  brachial  plexus,  or 
the  nerve-roots  ;  certain  nerves  are  also  particularly  prone  to  suffer  in 
peripheral  neuritis.  The  inflammation  of  the  plexus,  which  is  a 
unilateral  perineuritis,  is  separately  described  (Brachial  Neuritis)  ; 
the  latter  is  considered  in  the  general  account  of  Multiple  Neuritis. 
Secondary  neuritis,  set  up  by  injury  or  by  adjacent  inflammation,  may 
extend  upwards,  and,  reaching  the  plexus,  spread  to  other  nerves. 
Neuromata  are  rare.  Yarious  morbid  processes  in  the  neck  may  involve 
the  nerve-roots. 

Posterior  Thoracic  Nerve — Nerve,  to  the  Serratus. — In  consequence 
of  its  position  and  long  course,  the  nerve  to  the  serratus  often  suffers. 
After  being  formed  in  the  substance  of  the  scalenus  medius  muscle, 
by  the  union  of  branches  of  the  fifth  and  sixth  cervical  nerves,  it  passes 
behind  the  brachial  plexus  and  along  the  side  of  the  chest  to  the 
lower  border  of  its  muscle.  It  is  in  the  neck  that  the  nerve  is  most 
often  injured  ;  injury  to  the  axilla  less  often  implicates  it.  It  may  be 
damaged  in  the  neck  by  direct  pressure — as  in  carrying  on  the  shoulder 
a  heavy  sharp-cornered  object— or  by  severe  muscular  efforts,  such  as 
carrying  a  weight  that  does  not  press  into  the  neck,  wielding  a  heavy 
hammer,  or  long- continued  exertion  with  the  raised  arm,  as,  for  instance, 
in  whitewashing  a  ceiling.  In  such  cases  a  traumatic  neuritis  is  set  up 
by  the  violent  compression  of  the  nerve  consequent  on  the  forcible  or 
prolonged  contraction  of  the  muscle  through  which  it  passes.    Whe» 


102  SPINAL    NERVES. 

the  onset  of  the  palsy  occurs  immediately  on  the  exertion,  a  paralysing 
injury  to  the  nerve  must  have  occurred,  vyhich  will  be  followed  by 
secondary  neuritis.  Falls  and  blows  on  the  neck  and  shoulder  are 
occasional  causes  ;  there  is  usually  much  bruising  of  the  adjacent  parts. 
Punctured  and  gun-shot  wounds  occasionally  involve  the  nerve,  which 
may  also  be  affected  in  or  after  typhoid  fever,  influenza,  and  diphtheria. 
The  least  frequent  cause  is  exposure  to  cold,  such  as  a  draught  on  the 
neck,  or  sleeping  on  damp  earth.  I  have  once  known  it  to  follow  par- 
turition, developing  four  days  afterwards,  doubtless  from  neuritis 
excited  either  by  the  muscular  exertion  or  by  exposure  of  the  neck  to 
cold  during  the  labour.  An  hysterical  paralysis  in  the  distribution  of 
the  nerve  has  been  described  by  Yerhoogen.  The  serratus  suffers  also 
in  central  disease,  chiefly  in  progressive  muscular  atrophy  and  dys- 
trophy and  infantile  paralysis ;  but  in  these  it  is  always  associated  with 
palsy  of  other  muscles.  Isolated  paralysis  of  these  nerves  is  nine 
times  as  frequent  in  men  as  in  women.  It  occurs  chiefly  in  muscular 
workers,  and  duriag  the  active  period  of  life,  twenty-five  to  forty. 
The  immediate  causes  sufficiently  explain  this  relation  to  age  and  sex, 
and  they  also  account  for  another  fact — that  it  is  far  more  frequent  on 
the  right  side  than  on  the  left.  It  is  sometimes  bilateral,  but  the  two 
nerves  are  never  damaged  at  the  same  time.  One  man  was  affected  on 
the  right  side  after  carrying  heavy  beams  on  the  shoulder ;  he  then 
carried  them  on  the  left  shoulder,  and  the  left  serratus  became  paralysed. 

Severe  neuralgic  pains  in  the  neck  and  about  the  shoulder  commonly 
precede  and  accompany  the  onset  of  the  affection  when  due  to  neuritis. 
The  symptoms  of  the  paralysis  of  the  serratus  have  been  already  de- 
scribed (p.  35) .  It  is  readily  recognised  by  the  recession  of  the  posterior 
edge  of  the  scapula  from  the  thorax  when  the  arm  is  put  forwards 
(Fig.  8,  p.  36).  In  severe  cases  the  muscle  is  found  to  have  lost 
f  aradic  irritability,  although  it  may  still  contract  to  the  voltaic  current. 

The  course  of  a  severe  case  is  always  tedious.  Months  may  pass 
before  improvement  is  manifested,  and  the  paralysis  is  sometimes  per- 
manent. In  treatment  it  is  desirable  to  maintain  the  nutrition  of  the 
muscle,  as  far  as  possible,  by  electrical  stimulation,  and  its  superficial 
situation  renders  this  easy.  If  counter-irritation  is  desirable,  it  should 
be  applied  over  the  position  of  the  scalenus,  since  the  lesion  of  the 
nerve  is  commonly  at  this  place.  The  use  of  the  arm  should  be 
limited.  When  practicable,  the  elbow  should  be  carried  in  a  sHng  of 
eiich  a  length  as  slightly  to  raise  the  shoulder.  AH  movements  that 
may  involve  contraction  of  the  scalenus,  i.  e.  all  movements  needing 
elevation  of  the  shoulder,  must  be  prohibited. 

The  supra- scapular  nerve  arises  from  the  trunk  formed  by  the 
union  of  the  sixth,  fifth,  and  a  branch  of  the  fourth  cervical  nerves, 
but  its  fibres  come  from  the  fifth  and  partly  from  the  fourth.  It  ia 
occasionally  damaged,  alone  or  with  the  circumflex,  in  dislocation 
of  the  humerus,  and  by  falls  on  the  shoulder,  and  by  carrying 
heaw  weights.     In  some  cases  the  injury  may  be  to  the  upper  part 


NEBVKS    OF    THE    ARM.  103 

of  the  plexus  (see  p.  Ill),  which  may  recover  except  this  nerve  It 
suffered  with  the  circumflex,  in  a  case  recorded  by  Eemak,  from  acute 
limited  inflammation  of  the  plexus,  due  to  exposure  to  cold  after  over- 
use of  the  arms.  Bernhardt  has  seen  it  following  a  faU  upon  the 
hand.  The  result  of  its  disease  is  palsy  of  the  supra-  and  infra- 
spinatus (p,  37).  The  latter  is  the  more  important,  and  causes  a 
defect  of  the  rotation  outwards  of  the  humerus,  interfering  with  many 
movements,  and,  among  others,  with  the  movement  of  the  pen  along 
the  line  in  writing.  Undue  work  is  thrown  on  the  posterior  part  of 
the  deltoid  and  the  teres  minor;  the  deltoid  may  become  hypertrophied, 
and  its  posterior  edge  is  conspicuous  against  the  wasted  infra- spinatus. 
The  scapula  is  rotated,  so  that  the  lower  angle  is  moved  upwards  and 
inwards.  The  paralysis  of  the  supra- spinatus  is  unimportant,  except 
that  the  humerus  is  less  firmly  fixed  and  the  deltoid  more  readily 
fatigued.  It  can,  in  very  slight  degree,  supplement  the  deltoid,  and 
if  paralysed  with  the  deltoid,  the  head  of  the  humerus  falls  more  than 
it  does  when  the  deltoid  is  paralysed  alone.  Some  anaesthesia  over 
the  scapula  often  results  from  disease  of  this  nerve ;  early  in  the  affec- 
tion there  is  frequently  severe  pain  in  the  region  of  the  shoulder-girdle. 
The  Circumflex  nerve,  although  arising  from  the  posterior  cord  of 
the  plexus,  seems  to  derive  its  fibres  from  the  same  source  as  the 
supra- scapular,  the  fourth  and  fifth  cervical  nerves.  It  supplies  the 
deltoid  and  teres  minor,  and  the  skin  over  the  deltoid.  The  course 
of  the  nerve  renders  it  very  liable  to  injury  from  dislocations  and  falls 
on  the  shoulder,  and  from  the  pressure  of  a  crutch.  Simple  neuritis 
and  so-called  "  rheumatic  palsy  "  are  rare,  but  the  nerve  has  been 
affected  in  some  acute  diseases,  possibly  from  pressure,  and  in  rheumatic 
fever,  perhaps  by  inflammation  extending  from  the  joint;  typhoid 
and  diabetes  may  also  give  rise  to  circumflex  paralysis.  It  suffers, 
with  other  nerves,  from  disease  of  the  upper  part  of  the  brachial 
plexus,  and  the  chief  symptom  is  paralysis  of  the  deltoid  (p.  36), 
which  abolishes  almost  all  power  of  raising  the  arm,  a  very  trifling 
degree  of  abduction  by  the  supra- spinatus  alone  remaining.  The 
slight  nerve- supply  that  the  fore-part  of  the  deltoid  receives  from  one 
of  the  anterior  thoracic  nerves  is  insufficient  to  mitigate  the  effect  of 
the  paralysis  of  the  circumflex,  although  it  may  maintain  slight  power 
of  voluntary  contraction  in  the  anterior  fibres,  and  this  sometimes 
gives  rise  to  an  erroneous  impression  that  the  paralysis  of  the  circum- 
flex nerve  is  incomplete.  The  effect  of  the  conjoiaed  palsy  of  the 
teres  minor  is  unimportant.  The  wasting  of  the  deltoid  causes  a 
change  in  the  shape  of  the  shoulder  (Figs.  7  and  9).  Sensation  may 
be  lost  in  the  skin  over  the  lower  part  of  the  muscle,  and  the  loss 
sometimes  extends  on  to  the  shoulder  (Fig.  52)  ;  Hitzig  pointed  out 
many  years  ago  that  the  anaesthetic  area  is  often  the  seat  of  vaso-  motor 
paralysis.  In  some  cases  there  is  no  anaesthesia,  even  when  the  muscle 
is  wholly  paralysed ;  we  have  seen  that  this  is  often  the  case  in  nerve 
lesions  (p.  78).    Adhesions  are  apt  to  form  in  the  shoulder- joint,  in 


104  SPINAL   NERVES. 

part  probably  the  result  of  tropMc  cbanges,  since  the  circumflex  suppHes 
tbe  articulation  as  well  as  tbe  cliief  muscle  that  moves  it. 

r,, ,,,  ,y  The  diagnosis  of  the  paralysis  of  the  circum- 

flex nerve  is  easy.     The  only  condition  readily 
\       mistaken  for  it  is  anchylosis  of  the  shoulder- 
/  '        joint  in  a  stout  individual,  in  whom  the  state 

of  nutrition  of  the  muscle  is  not  readily  per- 
ceived. The  risk  of  error  is  increased  by  the 
fact  that  arthritis  and  paralysis  may  result 
>  from  the  same  cause — a  fall  on  the  shoulder. 
\  Passive  movement  at  once  solves  the  problem. 
The  scapula  moves  with  the  arm  in  anchylosis, 
and  not  in  palsy.  Furthermore,  attempts  at 
voluntary  contraction  leave  the  muscle  flaccid, 
while  if  the  affection  is  in  the  joint  the  muscle- 
|i:ii-I  w^  •'  ^~_..ui|  fibres  beome  rigid  in  the  attempt  to  contract. 
Vi&.  52.— Disease  of  the  The  importance  of  examining  the  joint  is  very 
circumflex  nerve  from  great  in  all  cases  of  interference  with  move- 
pressure  neuritis  during  ^^^^  ^^  ^j^  shoulder.  A'  patient  had  an 
acute  illness  ;  wasting  ot  ^ 

deltoid.   Thedotte.iline  epileptic  fit  which  was  supposed  to  have  left 
mdic:)tcs  the  area  of  cu-   "brachial  monoplegia,  merely  because  he  fell  on 

taneous  ansesthesia.  ,^         iin  in-  j       p    ,^ 

the  shoulder,  and  the  movement  of  the  arm 
was  lessened  first  by  pain,  and  then  by  arthritic  adhesions.  In  dia- 
gnosing the  lesion  from  those  of  other  nerves,  the  electrical  reactions 
and  the  area  of  anaesthesia  may  be  important. 

The  Musculo-cutaneous  nerve,  supplies  the  chief  flexors  of  the  elbow 
and  the  skin  over  the  radial  side  of  the  forearm.  It  is  scarcely  ever 
paralysed  alone,  but  often  suffers,  with  other  nerves,  in  disease  of  the 
brachial  plexus.  In  the  few  recorded  cases  the  cause  has  been 
traumatic,*  but  in  Eemak's  case  of  jiaralysis  of  the  supra- scapular 
and  circumflex  nerves  on  the  right  side,  from  limited  neuritis  of  the 
plexus,  there  was  complete  palsy  of  this  nerve  on  the  left,  thought  to 
be  due  to  a  still  more  limited  inflammation  in  the  left  brachial  plexus.f 
The  symptoms  correspond  to  its  function ;  there  is  paralysis  of  the 
biceps  and  brachialis  anticus  (see  pp.  38,  39),  the  effect  of  which  is 
especially  conspicuous  when  the  arm  is  supinated  and  the  suj)inator 
longus  cannot  flex  the  elbow.  There  may  also  be  anaesthesia  of  the  radial 
side  of  the  forearm. 

The  Musculo-spiral  nerve  is  more  frequently  paralysed  alone  than 
any  other  nerve  of  the  arm.  Arising  from  the  jDosterior  cord  of 
the  brachial  plexus,  it  seems  to  derive  its  motor  fibres  from  all  the 
nerve-roots  that  enter  the  plexus  except  the  first  dorsal.  It  suppHes 
the  triceps,  all  the  muscles  on  the  back  of  the  forearm,  the  extensors 
of  the  wrist  and  fingers,  both  the  supinators,  also  the  skin  on  the 
radial  side  of  the  back  of  the  hand,  the  back  of  the  thumb,  index 

*  See  Bernhardt.  '  Erkrank.  peripher.  Nerven.,'  1897,  p.  329. 
t  '  Neur.  Ceutralbl.,'  1896,  p.  578. 


NERVES  OF   THE    ARM.  105 

finger,  and  lialf  the  middle  finger.  It  is  thus  the  extensor  nerve  of 
the  arm,  and  has  a  more  complete  relation  to  a  single  function  than 
is  common  among  the  nerves  of  the  limbs  ;  a  fact  that  has  given  rise 
to  diagnostic  error,  since  a  palsy  limited  to  a  single  function  suggests 
a  central  cause.  The  frequency  with  which  this  nerve  suffers  is  due 
to  its  course.  As  it  leaves  the  brachial  plexus  to  wind  round  the  bone, 
its  positio^i.  exposes  it  to  injury  in  dislocation  or  from  the  pressure  of 
a  crutch  ;  the  most  common  form  of  "  crutch  palsy  "  is  paralysis  of 
this  nen  3.  Lying  as  it  does  close  to  the  humerus,  it  is  readily  torn 
in  fracture,  or  compressed  by  callus,  and  it  suffers  gravely  from  even 
temporary  pressure  against  the  hard  bone.  Such  pressure  is  often 
exerted  during  sleep,  especially  that  induced  by  alcohol.  The  nerve 
may  be  paralysed  by  the  pressure  to  which  it  is  exposed  when  the 
body,  with  the  arm  beneath  it,  rests  on  the  ground  or  on  a  hard 
bed.  Thus  a  man  slept  all  night  on  a  bench,  lying  on  his  right 
side  with  his  arm  beneath  him,  and  woke  in  the  morning  with  this 
nerve  paralysed.  Curiously,  a  precisely  similar  event  had  occurred 
to  him  three  years  before.  In  other  cases  the  pressure  on  the  nerve 
is  that  of  a  hard  and  sharp  object  over  which  the  arm  is  placed 
diu-ing  sleep,  as  the  edge  of  a  chair  or  the  side  of  a  couch.  The 
Continental  custom  of  tying  together,  behind  the  body,  the  arms 
of  a  prisoner,  often  causes  paralysis  of  this  nerve,  sometimes  on  both 
sides.  In  Russia  it  is  not  uncommon  in  infants,  from  the  popular 
practice  of  binding  the  arms  to  the  body  and  then  laying  the  child  to 
sleep  on  its  side.  I  have  three  tiines  met  with  paralysis  from  a  violent 
contraction  of  the  triceps,  once  during  the  act  of  pulling  on  a  tight 
pair  of  boots,  once  from  throwing  a  stone  with  energy,  and  once  from 
grasping  a  lamp-post  to  avoid  a  fall  during  a  severe  attack  of  giddi- 
ness. In  each  the  nerve  was  at  once  completely  paralysed ;  and  in  the 
second,  in  which  the  palsy  was  severe,  a  bruised  appearance  was  ob- 
served over  the  lower  part  of  the  triceps.  Neuritis  due  to  cold  is  often 
assumed  to  be  a  cause,  and  is  possibly  sometimes  effective,  but  the 
exposure  has  usually  been  during  alcoholic  sleep,  and  it  is  probable  that 
the  paralysis  is  due  to  compression  rather  than  to  cold.  The  same 
inflaence  may  also  have  been  operative  in  cases  in  which  the  nerve  has 
been  paralysed  during  an  acute  disease.  Although  in  a  case  of  para- 
lysis during  typhus,  described  by  Bernhardt,  neuritis  was  found  after 
death,  this  was  at  the  spot  at  which  pressure  is  usually  effective,  and 
the  inflammation  was  probably  thus  excited.  Pressure  always  sets 
up  neuritis,  and  the  diagnosis  of  a  primary  neuritis  is  never  justified 
if  the  spot  affected  is  one  liable  to  compression.  Paralysis  of  this 
nerve,  with  others,  has  followed  the  use  of  an  Esmarch's  bandage ;  and 
a  partial  palsy  sometimes  results  from  the  habitual  or  even  occasional 
injection  of  ether  beneath  the  skin  of  the  posterior  part  of  the  arm. 

The  symptoms  of  disease  of  the  musculo- spiral  nerve  are  paralysis 
of  the  extensors  of  the  elbow  and  of  the  wrist,  the  long  extensors  of  the 
fingers  and  thumb,  and  the  supinators  (see  pp.  37 — 40).    All  these  are 


106 


SPINAL   NER7ES. 


paralysed  by  a  complete  lesion  of  the  nerve  near  the  brachial  plexus. 
When  the  lesion  is  near  the  middle  of  the  humerus,  as  it  is  in  most 
cases  of  compression,  the  triceps  generally  escapes,  but  not  always. 
The  supinator  longus  is  usually  paralysed,  but  escapes  if  the  lesion 
is  below  the  origin  of  its  branch,  and  may  also  escape  if  the  injury  to 
the  nerve  is  incomplete.  In  sleep  palsy  it  usuully  suffers,  but  I  have 
seen  it  unaffected,  in  a  case  in  which  the  ext.  carpi  radialis  was  also 
but  little  affected,  and  Bernhardt  has  noted  the  escape  of  the  supinator 
in  an  otherwise  complete  paralysis  from  dislocation  of  the  humerus. 
The  extensor  palsy  causes  characteristic  wrist-drop,  and  loss  of  the 
power  of  extending  the  first  phalanges  of  the  fingers  and  the  thumb 
(seep.  40).  There  is  sometimes  a  gradation  of  palsy  from  the  first 
finger,  in  which  it  is  least,  to  the  fourth,  in  Avhich  it  is  greatest  (Fig. 
53).     The  same  gradation  is  seen  in  lead  palsy,  and  other  forms  of 


Fig.  53. — Pnralysis  of  the  musculo-spiral  nerve;  maximum  extension  of 
wrist  and  fingers.  The  extension  of  the  fingers  progressively  diminishes 
from  the  first  to  the  fourth.     (From  a  photograph.) 

peripheral  neuritis.  It  is  not  easy  to  explain.  The  action  of  the 
flexors  is  feeble,  from  the  loss  of  antergic  support ;  in  most  cases  of 
complete  palsy  I  have  found  the  power  of  flexion  reduced  from  this 
cause  to  one  third  of  the  normal.    The  loss  of  the  power  of  supination 

is   a   grave  inconvenience. 

If    an    object   is   grasped 

firmly    the    arm    becomes 

pronated.        The     patient 

tries    to    compensate    for 

the   loss    by   putting   the 

elbow     against     the    side 

and  rotating  the  humerus. 

Fig.  54.— Prominence  at  back  of  hand  from  para-  The    pronators    may    ulti- 

lysisof  the  extensors.     Tlie  patient  was  suffering  ^g^^gj      become   shortened. 

from  wrist-drop  due  to  silver  poisoning  (see  vol.  ^ni  n      •  e      .t 

ii, "  Aro-yria ").  The    over-tiexion    or     the 

carpus,  and  its  deficient 
support  by  the  extensor  tendons,  lead  to  a  prominence  of  synovial 
sacs,  and  perhaps  of  the  bones,  at  the  back  of  the  carpus  (Fig.  54). 
In  severe  cases  the  muscles  waste,  and  the  maximum  circumference  of 


NEliVES    OP   THE    ARM.  107 

the  limb  below  tlie  elbow  may  be  a  quarter  or  balf  an  incli  less  than 
on  the  other  side ;  at  the  same  time  there  may  be  thickening  of  the 
tendon-sheaths  at  the  back  of  the  hand.  The  electrical  reaction 
depends  on  the  severity  of  the  lesion  of  the  nerve ;  commonly  there 
is  well-marked  degenerative  reaction.  The  affection  of  sensibility  in 
the  area  supplied  by  the  nerve  is  very  variable.  The  skin  of  the 
upper  arm  rarely  loses  feeling ;  in  the  hand  sensation  may  be  normal 
although  the  muscular  paralysis  is  complete.  There  may  be  sub- 
jective "tingling"  in  the  part  although  there  is  no  loss  of  sensibility. 
The  elbow-  and  wrist-jerks  may  be  lost  even  when  the  reaction  of 
degeneration  is  not  present. 

The  diagnosis  is  easy  in  most  cases.  The  affection  is  distinguished 
from  lead  palsy  by  its  common  limitation  to  one  arm,  by  the  affec- 
tion of  the  supinator,  and  often  by  the  sudden  onset ;  that  from  lead 
is  gradual.  The  latter  is  further  characterised  by  the  early  onset  of 
the  degenerative  reaction  in  the  affected  muscles.  These  characters, 
and  the  commonly  obtrusive  cause,  always  suffice  for  the  diagnosis. 
It  must  be  remembered,  however,  that  this  nerve  is  one  most  fre- 
quently affected  in  many  other  forms  of  multiple  neuritis  (q.  v.). 
The  prognosis  in  disease  of  the  musculo-spiral  nerve  depends  on  the 
severity  of  the  lesion,  as  indicated  by  the  electrical  rea,ction.  When 
there  is  evidence  of  nerve  degeneration  the  paralysis  usually  lasts  for 
some  months.  Eecovery  ultimately  occurs  in  almost  all  cases.  The 
treatment  is  that  for  neuritis,  already  described,  but  attention  should 
be  paid  to  the  posture  of  the  limb,  so  as  to  avoid  tension  on  the 
affected  part  of  the  nerve.  For  this  reason,  and  also  because  strong 
contraction  of  the  triceps  may  further  injure  the  nerve,  extension  of 
the  elbow  should  be  avoided  in  cases  of  lesion  of  the  nerve  as  it  winds 
round  the  humerus. 

The  Median  nerve  supplies  the  pronators,  the  radial  flexor  of  the 
wrist,  the  palmaris  longus,  the  flexors  of  the  fingers  (except  the  ulnar 
half  of  the  deep  flexor),  the  muscles  that  abduct  and  flex  the  thumb, 
and  the  two  radial  lumbricales.  Its  motor  fibres  seem  to  have  an  exten- 
sive origin  from  all  the  cervical  roots  that  enter  the  brachial  plexus.  It 
subserves  sensation  on  the  radial  side  of  the  palm,  on  the  front  of  the 
thumb,  of  the  first  two  fingers,  and  of  half  the  third  finger,  and  also,  at 
least  in  many  persons,  on  the  back  of  the  last  phalanx  of  the  index  and 
middle  finger;  sometimes  also  on  the  adjacent  part  of  the  back  of  the 
ring  finger  and  the  back  of  the  last  phalanx  of  the  thumb.  Isolated 
palsy  of  this  nerve  is  not  frequent,  and  generally  results  from  wounds 
of  the  forearm,  or  fractiu^es  of  the  forearm  bones,  rarely  from  injuries 
in  the  upper  arm.  The  nerve  is  occasionally  the  seat  of  neuritis, 
which  may  be  induced  by  compression,  especially  in  those  who,  in 
their  work,  have  to  hold  an  object  between  the  hand  and  upper  arm. 
Occupation  paralyses  of  this  nerve  have  been  observed  in  ironers, 
locksmiths,  joiners,  cigarette  makers,  and  once  in  a  dentist.  The 
nerve  may  also  suffer  as  the  result  of  pressure  of  the  head  of  the 


108  SPINAL   NEEVES. 

humerus  in  sleep  or  narcosis,  while  in  former  days  it  was  not  infre- 
quently injured  by  unskilful  venesectors.  I  have  known  it  to  be 
paralysed  at  the  wrist  a  few  hours  after  a  severe  sprain  of  this 
joint.  Webber  has  recorded  a  case  in  which  it  seemed  to  be  injured 
by  a  violent  contraction  of  the  pronator  teres.  When  there  is  com- 
plete damage  to  the  nerve,  pronation  is  impossible  beyond  the  mid- 
position,  to  which  the  supinator  longus  can  bring  the  forearm,  and 
an  attempt  is  made  to  supplement  this  by  rotating  the  humerus 
inwards,  and  separating  the  elbow  from  the  side.  The  wrist  can  only 
be  flexed  with  a  strong  inclination  towards  the  ulnar  side.  The 
thumb  is  in  persistent  extension  and  adduction  (like  the  thumb  of  the 
ape,  Figs.  22  and  23,  p.  44),  and  cannot  be  opposed  to  the  tips  of  the 
fingers.  The  power  of  flexing  the  second  phalanges  on  the  first  is  lost, 
and  also  that  of  flexing  the  distal  phalanx  of  the  first  and  second 
fingers,  but  this  phalanx  can  still  be  flexed  in  the  third  and  fourth 
fingers  by  the  ulnar  half  of  the  flexor  profundus  (see  p.  41).  Inter- 
osseal  flexion  of  the  first  phalanx  is  still  possible,  and  the  unopposed 
extensor  action  of  the  interossei  on  the  middle  and  distal  phalanges 
tends  to  cause  a  subluxation  of  the  articulations  concerned.  The 
wasting  of  the  thenar  muscles  is  usually  conspicuous.  The  resulting 
condition  is  very  characteristic ;  but  if  the  damage  is  at  the  wrist,  flexion 
of  the  wrist  and  distal  phalanges  remains.  The  sensory  loss  is  variable ; 
it  may  be  absolute  or  absent.  If  there  is  anaesthesia  it  is  greater  on 
the  palmar  surface,  and  will  often  be  found  also  on  the  dorsal  aspect 
of  the  extremities  of  the  first  two  fingers.  Trophic  changes  of  the 
skin  and  nails  are  not  uncommon. 

The  Ulnar  nerve  comes,  through  the  inner  cord  of  the  plexus,  from 
the  last  cervical  and  first  dorsal  roots,  and  its  origin  from  the  lowest 
part  of  the  cervical  enlargement  gives  it  an  important  relation,  since  it 
is  the  first  of  all  the  brachial  nerves  to  be  affected  by  disease  that 
ascends  from  the  dorsal  to  the  cervical  region  of  the  spinal  cord.  The 
nerve  supplies  the  ulnar  flexor  of  the  wrist,  the  ulnar  half  of  the  deep 
flexor  of  the  fingers,  the  muscles  of  the  little  finger,  the  interossei, 
some  of  the  lumbricales,  the  adductor,  and  inner  head  of  the  short  flexor 
of  the  thumb.  Its  sensory  area  is  the  ulnar  side  of  the  hand,  back 
and  front,  generally  more  extensive  on  the  back  (two  fingers  and  a 
half)  than  on  the  front  { one  finger  and  a  half) .  The  course  of  the 
nerve,  superficial  behind  the  elbow  and  at  the  wrist,  renders  it  liable 
to  separate  injury.  It  is  often  implicated  in  wounds  of  the  forearm, 
and  about  the  elbow-joint,  by  dislocations  of  the  shoulder  and  elbow, 
enlargement  of  the  elbow  bones,  and  by  fractures  of  the  bones  of  the 
forearm.  It  is  occasionally  the  seat  of  neuritis,  and,  like  the  musculo- 
spiral  nerve,  it  is  sometimes  paralysed  by  pressure.  At  the  elbow  the 
projecting  bones  preserve  the  nerve  from  direct  pressure ;  although 
Duchenne  describes  paralysis  in  men  who,  while  at  work,  rest  the  bent 
•elbow  on  a  hard  substance,  this  mechanism  is  certainly  rare.  It  is  much 
more  common  for  the  nerve  to  suffer  in  long- continued  flexion  of  the 


NERVKS    OF    THE    ARM.  109 

elbo-w,  without  external  pressure;  and  from  this  cause  the  nerve  is 
sometimes  paralysed  during  sleep.*  I  have  three  times  seen  a  sleep 
palsy  of  the  ulnar  nerve.  The  tension  may  set  up  neuritis,  and  is 
especially  effective  if  the  resistance  of  the  tissues  to  morbid  influences 
is  lessened  by  general  ill-health.  Many  persons  must  have  noticed 
that  if,  when  out  of  health,  they  sleep  with  the  elbow  bent,  they  wake 
with  tingling,  and  even  loss  of  feeling,  in  the  region  supplied  by  the 
ulnar  nerve,  although  this  may  not  happen  when  they  are  in  good 
health.  If  the  ill-health  is  profound,  as  in  an  acute  illness,  local 
neuritis  of  considerable  intensity  may  be  set  up.  Thus  a  lady,  a  few 
days  after  childbirth,  who  was  prostrated  by  a  long,  exhausting  labour, 
noticed  tingling  in  the  side  of  the  hand,  and  whenever  she  bent  her 
elbow,  a  sensation  "  as  if  she  had  knocked  her  funny-bone."  Paralysis 
of  the  muscles  supplied  by  the  ulnar  nerve  followed  in  a  few  hours, 
and  in  a  fortnight  there  was  distinct  wasting.  When  I  saw  her,  six 
months  later,  the  ulnar  nerve  behind  the  elbow  was  distinctly  thickened. 
She  had  had  a  similar  but  more  transient  attack  after  a  previous  con- 
finement. A  similar  palsy  has  occurred  during  the  course  of  typhoid 
fever,  and  both  nerves  have  been  known  to  suffer  (Bernhardt).  In 
such  conditions  not  only  is  the  tissue  health  lowered,  but  warning 
sensations  are  often  unperceived.  Actual  cysts  of  the  nerve  have  been 
described  by  Bowlby  and  zum  Busch. 

The  importance  of  the  interosseal  flexion  of  the  fingers  for  many 
actions,  such  as  writing,  renders  palsy  of  the  ulnar  nerve  peculiarly  dis- 
abling. Several  illustrations  of  the  svmptoms  have  already  been  given 
(pp.  42  and  43).  In  flexion  of  the  wrist  the  hand  deviates  towards  the 
radial  side,  and  persistent  distortion  may  take  place.  Adduction  of 
the  thumb  is  lost,  and  so  are  most  movements  of  the  little  finger.  The 
fingers  cannot  be  flexed  at  the  first  or  extended  at  the  other  joints, 
but  the  loss  is  slighter  in  the  first  two  fingers  than  in  the  others,  because 
their  lumbricales  are  supplied  by  the  median  nerve.  In  time  the 
opponents  of  the  interossei,  by  their  contracture,  lead  to  over-exten- 
sion of  the  first  phalanges  and  flexion  of  the  others ;  the  "  claw-like 
hand  "  is  produced  (see  p.  43),  but  this  is  less  complete  than  in  cases 
of  progressive  muscular  atrophy,  on  account  of  the  escape  of  the  first 
two  lumbricales.  When  the  palsy  is  imperfect  it  may  be  possible  to 
extend  the  second  and  third  phalanges,  if  the  first  can  be  over-extended 
and  an  advantage  thus  given  to  the  interossei  by  the  lengthening  of 
the  course  of  their  tendons,  just  as  in  partial  paralysis  of  the  long 
extensors  they  may  be  able  to  extend  the  digits  if  the  wrist  is  flexed, 
but  not  if  it  is  extended.  In  lasting  damage  the  hypothenar  eminence 
may  disappear,  the  palm  is  hollow,  the  thenar  muscles  that  remain 

*  H.  Eraun  ('  Deutsch.  med.  Wocli.,'  1894)  believes  that  in  sleep  the  nerve  is 
compri'ssed,  not  at  the  elbow  but  at  the  shoulder,  particularly  when  the  arm  is 
abducted  and  carried  backwards,  so  that  the  hands  are  crossed  behind  the  head.  A 
similar  effect  may  result  from  the  arm  being  held  in  this  position  in  an  operation, 
».g.  the  removal  of  a  breast. 


110  SPINAL  NEEVES. 

stand  out,  and  the  opponens  may  cause  a  slight  forw^ard  rotation  of 
the  thumb. 

The  loss  of  sensation,  as  in  the  case  of  the  other  nerves,  varies  much, 
both  according  to,  and  irrespectiye  of,  the  degree  of  lesion  of  the 
nerve.  Subjective  sensations  are  common,  with  and  without  anaes- 
thesia, and  those  who  have  had  neuritis  are  sometimes  unable  to  bend 
the  elbow  for  a  few  minutes  without  a  sensation  of  tingling  in  the 
area  supjDlied.* 

The  diagnosis  of  disease  of  the  ulnar  nerve  is  easy.  A  difficulty  is 
occasionally  caused  by  the  circumstance  already  mentioned,  that  dis- 
ease at  the  lowest  part  of  the  cervical  enlargement  may  cause  sym- 
ptoms limited  to  the  region  of  this  nerve,  but  a  knowledge  of  the  fact 
is  usually  sufficient  to  prevent  an  error .f 

Combined  Palsies  op  the  Nekves  of  the  Arm. — ^Paralysis  in  the 
region  of  two  or  more  of  the  nerves  of  the  arm  is  very  common,  and 
results  from  many  causes.  It  may  be  due  to  disease  of  the  spinal 
cord  or  of  the  nerve-roots  within  the  spinal  canal,  but  with  these  we 
are  not  now  concerned.  The  most  frequent  causes  outside  the  spinal 
canal  are  the  following: — (1)  Morbid  processes  in  the  neck,  affecting 
the  nerve-roots  outside  the  spine  or  the  upper  part  of  the  brachial 
plexus,  especially  growths  and  exostoses.  (2)  Diseases  of  the  plexus 
itself,  neuritis  or  injury,  dislocations  of  the  shoulder,  the  stretching  by 
a  sudden  wrench  of  the  arm,  if  or  actual  rupture  of  the  nerves  entering 
the  plexus  by  great  local  violence.  §  (3)  Fractures  of  the  bones.  (4) 
Neuritis,  which  may  ascend  a  nerve  of  the  arm  to  the  part  of  the  plexus 
whence  it  comes,  and  there  spread ;  or  neuritis  of  the  plexus  may  be 
primary  and  of  various  extent,  "neuritis  migrans." 

The  last,  ascending  neuritis,  occurs  when  an  injury  of  a  single  nerve  ia 
followed  by  an  extension  of  the  motor  and  sensory  symptoms  to  the 
regions  supplied  by  other  nerves.  This  can  only  be  explained  by 
an  ascending  neuritis  reaching  the  junction  of  the  nerves  at  the 
brachial  plexus,  and  there  spreading,  of  ten  in  what  seems  a  random 
manner.  Thus  a  lady  cut  her  wrist  so  as  to  leave  a  scar  an  inch  long, 
over  the  position  and  in  the  direction  of  the  median  nerve.  The 
injury  caused  paralysis  and  wasting  of  all  the  muscles  in  the  hand 

*  Atisesthesia  of  the  region  supplied  by  the  ulnar  and  external  popliteal  nerves  is 
a  not  infrequent  symptom  of  general  paralysis  of  the  insane  and  tabes.  In  the 
ulnar  it  was  first  described  by  Biernacki, '  Neurol.  Centralbl.,'  1894,  in  the  external 
popliteal  by  Sarbo.     See  Savbd  and  v.  Deky,  '  Pest.  Med.-Chir.  Presse,'  1897. 

+  Poncet  ('Comptes  Rcndus,'  1888)  describes  a  manual  paralysis  in  glass-blowers 
(hooked-band,  main  en  crochet)  which  closely  simulates  ulnar  palsy,  from  which  it 
is  to  be  distinguished  by  the  freedom  of  the  thumb  and  the  absence  of  aneesthesia. 

X  Bowlby,  loc.  cit.,  p.  225. 

§  Rieiler  ('  Munch,  med.  Wochenschr,,'  1893)  describes  a  paralysis  affecting 
mainly  the  circumflex  and  musculo-spiral  nerves  of  the  left  arm,  and  occurring 
especially  in  bricklayers  from  the  pressure  of  the  hod  upon  the  shoulder. 


NERVES   OF    THE    ARM.  Ill 

supplied  by  this  nerve.  This  palsy  was  followed  by  a  gradual  loss  of 
power,  with  diminished  faradic  irritability,  in  the  long  extensor  of  the 
fingers,  the  ulnar  flexor  of  the  wrist,  and  also  by  defect  of  sensation 
in  the  skin  supplied  by  the  ulnar  nerve.  Hence  the  neuritis  must 
have  ascended  to  the  plexus  and  there  have  spread  along  each  root  of 
the  median  nerve.  Again,  a  woman  cut  her  right  hand  with  a  broken 
stone  bottle,  along  the  hyiDothenar  eminence.  Three  weeks  after- 
wards the  muscles  of  the  hand  supplied  by  the  idnar  nerve  gradually 
became  paralysed,  with  wasting  and  loss  of  irritability,  and  in  the 
area  of  the  skin  supplied  by  the  nerve  there  was  first  tingling  and  then 
diminished  sensibility.  Burning  pain  spread  up  the  front  of  the 
forearm,  and  inner  side  of  the  upper  arm  to  the  axilla ;  all  the  fore- 
arm muscles  lost  power,  the  flexor  carpi  ulnaris,  and  the  flexor  sublimis 
digitorum  in  greatest  degree;  the  tingling  and  lessened  sensibility 
spread  to  the  fingers  supplied  by  the  median  nerve,  and  the  abductor 
pollicis  wasted.  In  this  case  also  there  must  have  been  an  ascending 
neuritis  of  the  ulnar,  spreading  to  the  median  at  the  brachial  plexus. 
The  extension  is  sometimes  only  in  the  neighbourhood  of  the  cause.  A 
whitlow  on  the  thumb  was  followed  by  extreme  wasting,  with  reaction 
of  degeneration  in  all  the  thenar  muscles  supplied  by  the  median. 
But  a  case  is  on  record  in  which  a  nodular  inflammation  commencing 
in  the  ulnar  nerves  apparently  spread  up  to  the  sympathetic* 

The  brachial  plexus  is  occasionally  the  seat  of  a  primary  neuritis. 
This  is  described  in  a  separate  section  (p.  119),  as  it  is  a  defined  and 
important  disease. 

In  dislocation  of  the  shoulder  the  displaced  head  of  the  humerus  may 
damage  the  nerves.  In  subcoracoid  dislocation  they  are  necessarily 
compressed.  The  extent  of  injury  varies  greatly ;  only  one  nerve  may 
suffer,  or  not  one  may  escape.  Every  muscle  of  the  arm  may  be  para- 
lysed, from  the  deltoid  downwards.  In  most  cases  the  injury  is 
severe  in  degree;  there  is  rapid  wasting  of  the  muscles,  with  the 
reaction  of  degeneration,  and  there  is  a  great  tendency  to  the  occiu-- 
rence  of  trophic  changes  in  the  skin.  It  was  in  a  case  of  this  character 
that  the  forearm  and  hand  were  covered  with  blisters  from  the  appli- 
cation of  water  that  seemed  merely  warm  to  a  healthy  hand  (p.  79). 

Fracture  of  the  humerus  may  damage  both  the  musculo -spiral  and 
ulnar  nerves,  rarely  the  median.  Fracture  of  the  bones  of  the  forearm 
often  injures  both  the  median  and  ulnar  nerves. 

Injuries  to  the  neck  sometimes  cause  a  partial  paralysis  of  the  arm 
of  peculiar  distribution,  the  special  characters  and  significance  of 
which  were  first  made  known  by  Erb.f  A  similar  paralysis  may 
come  on  apart  from  injury.  The  muscles  affected  are  the  deltoid, 
often  the  supra-spinatus  and  infra-spinatus,  the  biceps  and  brachialis 
•  Remak, « Deutsch.  Klinik,'  1864,  p.  159. 

t  Heidelberg  Society,  1874,  'Ziemssen's  Handbuch/  1874,  Bd.  xii,  pt.  2,  p.  509; 
Bee  also  Bernhardt, '  Zeitscb.  f.  kl.  Med.,'  1882,  Bd.  iv,  p.  415.  A  previous  descrip- 
tion by  Uui  lienne  in  1885  bad  escaped  notice. 


112 


SPINAL   NERVES. 


anticus,  and  tlie  supinators.  Erb  found  tliat  fhere  is  one  spot  between 
tlie  scaleni,  corresponding  to  tlie  sixtli  cervical  nerve,  at  wMcli  electrical 
stimulation  puts  all  tliese  muscles  ia  action.  Hoedemaker,  vrlio  has 
described  two  cases  of  this  palsy,  finds  the  motor  point  in  a  line  drawn 
from  the  sterno- clavicular  articulation  to  the  seventh  cervical  spine, 
1-5  centimetres  from  the  edge  of  the  trapezius.  The  palsy  is  appa- 
rently dependent  on  disease  of  the  roots  of  the  fifth  and  sixth  cervical 
nerves,  and  the  fifth,  it  will  be  remembered,  receives  a  twig  from  the 
fourth.*  Budingerf  and  Graupp|  have  recently  shown  that  in  forcible 
elevation  of  the  arm  some  backward  rotation  of  the  clavicle  occui-s, 


Ftp.  55. 


Fig.  ofi. 


Pi&.  57. 


Pig.  55. — Combined  palsy  of  deltoid,  snpra-spinatns,  and  infra-spinatns,  from  a  fall 

on  the  shoulder. 
Figs.  56,  57. — Left  hand  of  a  patient  suffering  from  a  growth  beside  the  lower  cervical 
spine  compressing  the  nerve-roots.  There  was  ansesthesia  of  all  parts  supplied 
by  the  brachial  and  cervical  plexus.  The  arm  was  adducted,  the  elbow  flexed,  the 
hand  in  the  posture  shown,  flexion  of  first,  extension  of  second,  strong  flexion 
of  last  phalanges,  the  first  phalanx  of  the  thumb  over-extended,  the  second  flexed. 
Rigidity  extreme,  and  an  attempt  to  overcome  it  caused  great  pain.  There 
was  also  some  contractare  of  the  foot  (equino-valgus). 

compressing  the  fifti  and  sixth  cervical  nerves  against  the  first  rib. 
The  fact  that  in  this  case  the  nerve-fibres  are  simply  contused  and  not 
broken  will  no  doubt  account  for  the  frequency  of  recovery  from  this 
form  of  paralysis.  Besides  injuries,  this  group  of  palsies  may  result 
from  non-traumatic  processes,  probably  from  neuritis,  and  also  from 

•  Erb  referred  the  palsy  to  disease  of  the  sixth  nerve.  The  experiments  of 
Perrier  and  Yeo  point  to  the  fifth  and  fourth  roots  as  those  innervating  these 
mnscles,  but  his  subsequent  correction  indicates  the  fifth  and  sixth  (see  p.  99,  foot- 
uote). 

t  '  Arch.  f.  klin.  Chir.,'  1894.  J  '  Centralblatt  f.  Chir.,'  1894. 


NERVES   OP   THE   AEM.  113 

growtlis  in  tM3  situation.  Dxich.enne,  Seeligmiiller,  and  otliers  have 
also  described  paralysis  of  similar  distribution  produced  in  infants 
during  birtb,  either  by  pressure  from  the  position  of  the  ai-m,  or  by 
traction  on  the  neck  with  the  finger  or  hook  during  turning.  Dis- 
location of  the  shoulder  during  delivery  may  cause  very  extensive 
damage  to  the  plexus.  Permanent  paralysis  of  each  arm  was  the 
result  of  bilateral  dislocation  caused  by  a  midwife  dragging  a  ehild 
out  by  the  two  arms.*  Most  obstetrical  cases  slowly  recover.  Those 
due  to  injury  in  adult  life  are  often  severe ;  the  symptoms  continue 
for  a  long  time,  and  may  be  permanent. 

A  paralysis  complementary  to  that  of  Erb,  in  which  the  muscles 
escape  which  suffer  in  that  form,  results  from  disease  of  the  nerve- 
roots  below.f  It  is  often  associated  with  ocular  symptoms,  such  as 
narrowing  of  the  pupil  and  palpebral  fissure. 

Morbid  processes  connected  with  the  bones  of  the  cervical  spine 
sometimes  produce  combined  symptoms  of  irritation  and  palsy  of 
the  nerves  of  the  arm,  pain,  hypersesthesia,  ansesthesia,  paralysis  of 
muscles,  and  extreme  muscular  contractures,  often  very  irregular  in 
distribution  (such  as  are  shown  in  Figs.  56,  57).  I  have  several  times 
known  sensory  symptoms  in  the  arm  to  be  due  to  a  chronic  syphilitic 
celliditis  beside  the  vertebrae,  causing  deep-seated  induration  and 
severe  compression  of  the  nerves ;  in  each  case  it  was  completely 
removed  by  antisyphilitic  treatment.  Growths  at  the  part  may  also 
compress  the  subclavian  artery,  and  the  weaker  pulse  may  aid  the 
diagnosis.^ 

DiAQNOSis. — The  diagnosis  of  diseases  of  the  nerves  of  the  arm  has 
been  for  the  most  part  sufficiently  considered  in  connection  with  the 
special  nerves.  It  consists  chiefly  in  an  application  of  the  facts  there 
stated.  One  or  two  more  general  considerations  deserve,  however, 
a  brief  mention.  Some  diseases  of  the  spinal  cord  are  first  manifested 
in  the  arm,  by  muscular  palsy,  wasting,  or  by  ansesthesia.  The  risk 
of  error  is  prevented,  in  most  cases,  by  the  absence  of  any  corre- 
spondence between  these  symptoms  and  the  functions  of  special 
nerves,  by  the  absence  of  any  indication  of  morbid  processes  in  the 
neighbourhood  of  the  nerves,  and  by  the  presence  of  other  signs  of 
disease  of  the  spinal  cord.  It  has  been  mentioned  that  disease  in  the 
lowest  part  of  the  cervical  enlargement  may  be  manifested  only  in  the 
region  of  the  ulnar  nerve,  the  lowest  in  origin  of  all  the  brachial 
nerves.  Such  disease  is,  however,  usually  bilateral,  and  secondary  to 
disease  in  the  dorsal  region  of  the  cord,  the  indications  of  which  have 

•  Danchez,  *  Ann.  de  Gyn.,'  &c.,  Oct.,  1891.  Double  palsy  in  adults  is  occasioaally 
Been,  as  in  a  case  recorded  by  Bernhardt,  in  which  the  arms  were  pulled  on  for  an 
hour  to  steady  tlie  patient  during  the  operation  of  oSphorectomy. 

t  It  has  been  termed  "  Klumpke's  paralysis,"  because  investigated  in  Vulpian's 
laboratory  by  Mdlle.  Klumpke  in  1883  (see  •  Zeitsch.  f.  Nervenh.,'  1891)  ;  it  had, 
however,  been  described  long  previously,  notably  in  1827  by  Flaubert. 

J  Bowlby,  loc.  cit. 

VOL.  I.  8 


114  SPINAL  NERVES. 

preceded  the  symptoms  in  tlie  arm.     A  knowledge  of  these  facts,  an 
a  careful  consideration  of  the  distribution  and  course  of  the  symptoms, 
will  rarely  leave  the  observer  in  doubt. 

Treatment. — There  is  little  in  the  treatment  of  the  diseases  of  the 
nerves  of  the  arm  that  requires  special  mention.  The  chief  measure 
is  the  treatment  of  the  cause  of  the  paralysis.  Any  present  source  of 
pressure  must  be  removed  as  far  as  possible.  The  ends  of  a  divided 
nerve  must  be  sutured.  It  is  remarkable  how  quickly  this  has  some- 
times been  followed  by  the  restoration  of  conducting  power.  The 
posture  of  the  arm  must,  in  all  <  ases,  be  such  as  to  avoid  tension  on 
an  injured  or  inflamed  nerve.  The  treatment  of  neuritis  is  described 
in  the  next  section.  Electrical  stimulation  of  the  muscles  is  of  great 
importance  in  a.ll  cases  of  severe  damage  to  the  nerves.  Even  in 
old  and  stationary  cases  it  sometimes  starts  some  improvement. 

Nerves  of  the  Lowek  Limb. 

Disease  of  these  nerves  is  far  less  common  in  the  lower  than  in  the 
upper  limb,  with  one  important  exception, — the  primary  disease  of  the 
sciatic  nerve  that  goes  by  the  name  of  "  sciatica."  This  affection  is 
reserved  for  separate  description. 

The  nerves  of  the  leg  are  derived  from  the  lumbar  and  sacral 
plexuses.  The  lumbar  plexus,  consisting  of  the  first  three  and  a  half 
lumbar  roots,  supplies  the  skin  of  the  lower  part  of  the  abdomen,  of 
the  front  and  sides  of  the  thigh,  of  the  inner  side  of  the  lower  leg 
and  foot.  It  supplies  also  many  muscles — the  cremaster,  those  that 
flex  and  adduct  the  hip-joint,  and  those  that  extend  the  knee.  Its 
branches  for  the  leg  are  the  obturator  and  anterior  crural  nerves. 
The  sacral  plexus  consists  of  the  fifth  lumbar  root  and  half  the  fourth 
(lumbo-sacral  cord)  and  the  first  four  sacral  nerves,  of  which,  however, 
only  the  upper  three  have  to  do  with  the  leg.  This  plexus  innervates 
the  extensors  and  rotators  of  the  hip,  the  flexors  of  the  knee,  and  all 
the  muscles  that  move  the  foot,  together  with  the  skin  of  the  gluteal 
region,  the  back  of  the  thigh,  the  outer  side  and  back  of  the  lower  leg, 
and  most  of  the  foot.  Its  chief  nerves  are  branches  to  the  outward 
rotators  of  the  hip,  the  gluteal  nerve,  and  the  small  and  great  sciatic. 

The  results  obtained  by  Ferrier  and  Yeo  (see  p.  99)  on  stimulating  the  roots 
o£  the  lumbar  and  sacral  plexuses  in  the  monkey  may  be  thus  summarised : 
Lumbar  I  and  II.  Lower  abdominal  muscles  (not  cremaster). 

III.  Psoas  and  iliacus,  sartorius,  extensors  of  knee;  (flexion  of  hip  and 

extension  of  leg). 
IV.  Glutei,  adductors,  extensor  cruris,  peroneus  longus ;  (extension  of 

hip  and  knee,  elevation  of  outer  side  of  foot). 
V.  Glutei,  hamstrings,  and  all  the   muscles  in  front  and  back   of 
lower  leg ;  (rotation  of  thigh  outwards,  flexion  and  rotation  inwards 
of  leg ;  extension  of  foot  with  elevation  of  outer  edge  ;  flexion  of 
distal  phalanges  of  toes). 


NJSEVJiS    OF    THE    LEG.  llJ> 

Sacral  I.  Hamstrings,  calf  mnscles,  long  flexor  of  toes,  intrinsic  muscles  of 
foot;  (slight  outward  rotation  of  thigh,  flexion  of  knee,  extension 
of  foot,  adduction  of  great  toe,  flexion  of  first  phalanges  of  all  toe» 
and  of  both  phalanges  of  great  toe). 
II.  Intrinsic  muscles  of  foot;  ("  interosseal "  flexion  of  toes,  similar  to 
the  last). 
These  results  cannot  be  simply  applied  to  man.     For  instance,  it  is  certain 
that  in  man,  one  flexor  of  the  hip,  the  psoas,  and  the  cremaster,  are  largely 
innervated  from  the  second  lumbar,  but  no  indication  of  the  action  of  either  of 
these  could  be  observed  on  stimulating  this  root  in  the  monkey. 

The  distribution  of  the  sensory  fibres  of  the  nerve-roots  is  considered  in  the 
chapter  on  the  Spinal  Cord. 

The  Lumbar  plexus  itself  is  sometimes  damaged  by  growths  in  the 
abdomen  (especially  by  those  that  spring  from  the  lumbar  glands)  and 
by  psoas  abscess,  while  the  nerve-roots  may  suffer  in  disease  of  the 
bones  of  the  vertebrae  and  in  meningitis.  The  plexus  may  also  be  in- 
vaded by  inflammation  ascending  the  lumbo- sacral  cord  from  the  sacral 
plexus,  and  it  is  occasionally  the  seat  of  spontaneous  neuritis.  The 
obturator  nerve  is  rarely  affected  alone ;  it  has  been  sometimes  damaged 
in  the  course  of  parturition.  The  anterior  crural  nerve  has  suffered 
from  the  same  cause,  and  is  sometimes  injured  by  wounds  of  the  groin 
or  thigh,  or  by  dislocation  of  the  hip-joint.  Symptoms  may  be  limited 
to  it  in  disease  of  the  nerve-roots,  and  as  in  injury  or  growths  of  the 
vertebrae,  its  paralysis  occasionally  follows  acute  or  subacute  inflam- 
mation of  the  knee-joint. 

Of  the  ijiterf erence  with  movement  that  may  result,  the  paral}  sis  of 
the  flexors  of  the  hip  depends  on  the  position  of  the  disease.  This 
paralysis  is  total  only  when  the  lumbar  plexus  is  damaged.  Disease  of 
the  anterior  crural  nerve,  within  the  abdomen,  does  not  affect  the  psoas, 
but  may  paralyse  the  iliacus,  and  so  weaken,  without  abolishing,  the 
power  of  flexion.  The  chief  symptom  of  disease  of  this  nerve  is  the 
loss  of  power  in  the  extensors  of  the  knee,  and  the  wasting  of  these 
muscles,  together  with  loss  of  the  knee-jerk  which  results  from  the 
interruption  of  the  reflex  arc.  The  effect  of  these  palsies  on  movement 
is  very  serious  (see  pp.  46,  47).  Anaesthesia  involves  the  whole  of  the 
thigh  (except  a  strip  along  the  middle  of  the  back),  the  inner  side  of 
the  leg  and  foot,  and  the  adjacent  side  of  the  first  and  second  toes.  In 
many  cases  the  paralysis  is  incomplete,  and  the  symptoms  present 
corresponding  variation.  Irritation  of  the  nerve  may  cause  severe 
pain  in  the  region  supplied  by  it.  This  is  sometimes  an  early  symptom 
of  a  growth  near  the  spine.  The  pain  in  neuritis  of  the  plexus  may 
extend  along  the  course  of  the  ilio-hypogastric,  ilio-inguinal,  and  genito- 
crural  nerves,  to  the  lower  part  of  the  abdomen  and  groin. 

Impairment  of  sensation  in  an  area  on  the  front  of  the  lower  half  of 
the  thigh  is  occasionally  met  with  as  an  isolated  symptom,  usually  in 
men  in  the  second  half  of  life.  It  comes  on  without  pain,  and  may  pass 
away  after  months,  or  may  persist  for  years.     The  loss  is  greatest  in  the 


116  SriNAL  NERVES 

middle  of  the  region,  but  the  transition  to  nonnal  sensibility  is  well 
defined ;  its  origin  and  nature  are  mysterious,  except  that  the  subjects 
of  it  are  usually  gouty.    It  seems  to  have  little  significance. 

Paralysis  of  the  obturator  nerve  causes  a  loss  of  the  power  of  adduc- 
tion of  the  thigh,  so  that  the  patient  cannot  put  one  leg  across  the 
other.  Rotation  of  the  thigh  outwards  is  interfered  with,  but  in 
slighter  degree  than  adduction.  The  effect  of  these  palsies  (described 
on  p.  47)  is  far  less  serious  than  is  the  disability  which  results  from 
disease  of  the  anterior  crural.  There  is  disturbance  of  sensation  along 
the  inner  side  of  the  thigh  and  knee  ;  an  obturator  hernia  may  press 
upon  the  nerve,  giving  rise  to  paresis  of  the  muscles  which  it  supphes. 
and  burning  pains  in  the  region  of  its  sensory  distribution. 

The  superior  gluteal  nerve  occupies  an  intermediate  position  between 
the  two  plexuses,  arising  as  it  does  from  the  lumbo-sacral  cord,  which 
descends  from  the  fourth  and  fifth  lumbar  roots.  Its  disease,  which 
is  very  rare  in  isolated  form,  causes  paralysis  of  the  gluteus  medius 
and  minimus,  with  a  loss  of  abduction  and  circumduction  of  the  thigh 
(see  pp.  45,  46). 

The  Sacral  plexus  is  sometimes  damaged  by  growths  in  the  pelvis, 
by  pelvic  inflammation  of  various  kinds,  and  by  compression  during 
parturition.  In  the  child  it  may  be  damaged  by  traction  on  the 
legs.  It  is  also  often  the  seat  of  neuritis,  which,  however,  less 
frequently  begins  in  the  plexus  than  ascends  to  it  from  the  sciatic 
nerve.  Apart  from  spontaneous  neuritis,  which  will  be  separately 
desmbed,  the  sciatic  nerve  outside  the  pelvis  is  occasionally  injured  by 
woiMids,  rarely  by  dislocations  of  the  hip,  often  by  disease  of  the  bone, 
and  by  adjacent  morbid  growths.  It  is  also  a  relatively  frequent 
seat  of  neuroma.  Of  the  terminal  branches,  the  external  popliteal,  by 
its  superficial  course,  and  proximity  to  the  hard  bone,  suffers  from 
traumatic  lesions  of  various  kinds ;  it  is  also  prone  to  spontaneous 
neuritis.  This  nerve  is  homologous  with  the  musculo- spiral  nerve  of 
the  arm,  and  presents  an  analogous  liability  to  disease  ;  it  occasionally 
suffers  in  persons  whose  occupations  entail  much  kneeling.  The  pos- 
terior tibial  nerve  is  more  secure  in  its  deeper  course,  but  may  be 
damaged  by  fracture  of  the  bones. 

The  symptoms  of  palsy  of  the  sciatic  nerve  vary  much  in  their 
character,  according  to  the  position  of  the  disease.  The  small  sciatic 
is  implicated  only  when  the  mischief  involves  the  pelvic  plexus,  and  it 
scarcely  ever  suffers  alone.  The  effect  is  palsy  of  the  gluteus  maximus, 
which  interferes  with  rising  from  a  seat  more  than  with  walking 
(see  p,  45).  There  is  also  anaesthesia  of  the  skin  in  the  middle  third 
of  the  back  of  the  thigh,  and  in  the  upper  half  of  the  caK,  A  lesion 
of  the  sciatic  nerve,  near  the  sciatic  notch,  paralyses  the  flexors  of  the 
leg  (which  are  also  extensors  of  the  hip  ;  seep.  47),  and  all  the  muscles 
below  the  knees.  Often  the  lesion  is  below  the  upper  third  of  the 
thigh,  and  then  the  flexors  of  the  leg  escape.  Even  when  all  the 
muscles  below  the  knee  are  paralysed,  walking  is  still  possible,  the  foot 


NERVES  OF  THE   LEG.  117 

being  raised  by  over-flexion  of  the  hip.  Th©  anaesthesia  that  results 
from  a  lesion  of  the  nerve  below  the  origin  of  the  small  sciatic,  involves 
the  outer  half  of  the  leg,  the  greater  part  of  the  dorsum  of  the  foot, 
and  all  the  sole,  but  the  leg  may  escape,  perhaps  through  union  "with 
other  nerves. 

The  symptoms  of  disease  of  the  branches  are  as  follows : 

That  of  the  external  popliteal  or  peroneal  nerve  causes  paralysis  of 
the  tibialis  anticus,  long  extensor  of  the  toes,  peronei,  and  extensor 
brevis  digitorum.  The  effect  of  this  is  a  loss  of  all  power  of  flexing 
the  ankle  and  of  extending  the  first  phalanx  of  the  toes  (see  p.  50). 
The  foot  cannot  be  raised  from  the  ground  in  walking,  and  talipes 
equinus  ultimately  results  (Fig.  30,  p.  51),  which  may  be  attended  with 
persistent  flexion  of  the  first  phalanges  of  the  toes  from  contracture  of 
the  ujiopposed  interossei.  There  is  also  anaesthesia  on  the  outer  half 
of  the  front  of  the  leg,  and  on  the  dorsum  of  tbe  foot.* 

Disease  of  the  internal  'popliteal  nerve  paralyses  the  popliteus,  calf 
muscles,  tibialis  posticus,  and  long  flexors  of  the  toes,  as  well  as  the 
muscles  of  the  sole.  In  addition  to  the  disability  which  characterises 
paralysis  of  the  plantar  muscles,  there  is  loss  of  the  inward  rotation  of 
the  flexed  leg  if  the  disease  is  so  high  as  to  involve  the  branch  to  the 
popliteus,  and  there  is  also  loss  of  the  power  of  extending  the  ankle- 
joint.  Talipes  calcaneus  resxdts  (Fig.  28,  p.  50).  The  sensory  loss 
is  on  the  outer  lower  part  of  the  back  of  the  leg,  and  on  the  sole,  but 
varies  much. 

The  plantar  nerves  rarely  suffer  alone.  A  lesion  of  the  internal  nerve 
causes  anaesthesia  on  the  inner  part  of  the  sole,  and  plantar  surface  of 
the  three  inner  toes  and  half  the  fourth,  together  with  paralysis  of  the 
short  flexor  of  the  toes,  the  plantar  muscles  of  the  great  toe  (except 
the  adductor),  and  of  the  two  inner  lumbricales. 

Disease  of  the  external  nerve  produces  anaesthesia  of  the  skin  on  the 
outer  half  of  the  sole,  the  Kttle  toe  and  half  the  fourth,  paralysis  of 
the  flexor  accessorius,  the  muscles  of  the  little  toe,  all  the  interossei, 
the  two  outer  lumbricales,  and  the  adductor  of  the  great  toe.  The 
effect  of  this  palsy  (see  p.  52)  is  serious,  since  the  toes  cannot  take 
their  proper  share  in  propelling  the  body  forward  in  walking,  and  they 
gradually  become  flexed  at  the  last  two  joints  and  extended  at  the 
others,  from  the  contracture  of  the  opponents  of  the  interossei, — ^a 
position  of  the  toes  that  causes  serious  inconvenience  in  walking. 

Diagnosis. — The  diagnosis  of  diseases  of  the  nerves  of  the  leg  is 
determined  by  the  same  general  principles  as  those  that  have  been 
mentioned  as  applicable  to  the  nerves  in  general,  and  to  the  nerves 
of  the  arm  in  particular.  The  limitation  of  the  symptoms  to  the 
functional  areas  of  individual  nerve-trunks,  the  evidence  afforded  by 
nutrition,  irritability,  and  reflex  action  that  the  muscles  are  separated 

*  Anaesthesia  in  the  district  supplied  by  this  nerve  may  be  a  symptom  of  various 
progressive  nervous  diseases;  it  was  first  described  in  this  connection  by  Sarbo  (see 
p.  110,  footnote). 


118  SPINAL   NERVES. 

from  the  spinal  cord,  tlie  implication  of  tlie  sensory  functions,  and 
often  tlie  tenderness  of  the  affected  nerres,  indicate,  in  most  cases, 
the  seat  of  the  disease.  To  these  signs  are  often  added  other  indica- 
tions of  a  local  cause,  corresponding,  in  its  position,  with  the  nerve 
to  which  the  symptoms  point. 

The  relation  of  nerve-trunks  to  nerve-roots,  although  by  no  means 
simple,  is  certainly  less  complex  in  the  case  of  the  nerves  of  the  leg 
than  in  those  of  the  arm.  This  is  especially  the  case  in  the  lumbar 
plexus,  and  it  leads  to  an  occasional  difficulty  in  diagnosis.  Pressure 
on  the  spinal  cord,  for  instance,  at  the  level  of  the  origin  of  the  fourth 
lumbar  roots,  may  cause  symptoms  identical  with  those  of  a  partial 
lesion  of  the  anterior  crural  nerve.  For  instance,  I  have  known  para- 
lysis of  the  extensors  of  the  knee,  loss  of  the  knee-jerk,  with  anaesthesia 
in  the  front  of  the  thigh,  to  be  the  result  of  a  gumma  at  the  side  of  the 
cord  at  this  level.  But  other  evidence  of  a  spinal  lesion  is  rarely  absent 
under  such  circumstances,  and  in  this  patient  a  foot-clonus,  due  to  the 
pressure  on  the  pyramidal  fibres,  left  no  doubt  as  to  the  situation  of  the 
disease.  Another  difficulty  arises  from  the  long  course  of  the  nerve-roots 
in  the  cauda  equina,  disease  of  which  may  simulate  that  of  the  nerves  of 
the  leg.  Implication  of  this  region  is  most  commonly  due  to  syphilis, 
trauma,  congenital  defects  (spina  bifida),  or  new  growths.*  But 
the  symptoms  are  commonly  bilateral  in  consequence  of  the  prox- 
imity of  the  nerve-roots  of  the  two  sides.  In  all  cases  in  which 
symptoms  are  bilateral  (unless  there  is  evidence  of  a  disease  known 
to  cause  symmetrical  lesions,  such  as  multiple  neuritis),  the  suggestion 
is  that  the  disease  is  situated  where  the  motor  or  sensory  paths  of 
each  side  are  so  near  that  they  can  be  affected  by  a  single  lesion,  i.  e. 
that  the  disease  is  within  the  spinal  canal.  But  here,  as  in  other 
cases,  we  cannot  reverse  our  diagnostic  rules.  Disease  of  the  spinal 
cord  does  not  always  cause  bilateral  symptoms.  A  limited  lesion  of 
one  anterior  cornu  may  be  so  placed  as  to  pa'ralyse  the  muscles  supplied 
by  a  single  nerve,  and  a  doubt  may  be  felt  as  to  the  central  or  peri- 
pheral origin  of  such  palsy.  The  muscles  supplied  by  the  anterior 
crural  nerve,  and  the  muscles  in  the  front  of  the  lower  leg  supphed 
by  the  external  popliteal,  are  those  of  which  the  central  palsy  most 
often  leads  to  doubt.  The  mode  of  onset,  the  presence  or  absence 
of  sensory  symptoms,  the  rarity  of  acute  spinal  palsy  except  in  child- 
hood, and  of  nerve-lesions  except  in  adult  life,  the  wider  initial  pre- 
valence of  the  palsy  in  acute,  and  its  later  extension  in  chronic, 
cornual  disease, — these  suffice  as  a  rule  to  remove  any  doubt. 

It  is  important  to  remember  that  the  pressure  of  a  growth  may 
cause  either  a  chronic  or  an  acute  affection  of  the  nerves.  The  chronic 
symptoms  result  from  compression ;  the  acute  from  a  neuritis  set  up 
by  the  pressure  and  irritation. 

Tkeatment. — The  treatment  of  disease  of  the  nerves  of  the  leg 

*  See  Thorbura,  *  Brain,*  1888 ;  Fr.  Schulze, '  Deutsche  Zeitschr.  XervenheilkV 
1894,  vol.  V. 


BRACHIAL   NEURITIS.  119 

does  not  differ  from  that  of  disease  of  the  nerves  of  the  arm.  More 
care,  perhaps,  is  needed  to  avoid  increasing  present  mischief,  or 
inviting  a  relapse,  by  exposure  to  cold  or  bj  fatiguing  exertion.  More 
care  is  also  needed  to  obviate  the  tendency  to  secondary  contractures  in 
the  case  of  palsies  of  long  duration,  and  in  those  attended  by  pain  in 
which  the  patient  seeks  ease  in  postures  to  which  the  muscles  only  too 
readily  adapt  themselves.  The  contraction  of  the  hamstrings,  from  con- 
stant flexion  of  the  knees,  occurs  very  readily  and  is  most  troublesome  ; 
that  of  the  calf  muscles,  which  occurs  when  the  flexors  of  the  ankle  are 
paralysed,  also  constitutes  a  serious  obstacle  to  walking  after  recovery. 
A  little  timgly  care,  by  attention  to  posture,  will  often  save  a  vast 
amount  of  later  trouble.  That  of  the  calf  muscles,  however,  which 
is  due  to  the  extension  produced  by  the  weight  of  the  foot  as  the 
patient  lies,  cannot  always  be  entirely  prevented,  but  may  be  lessened 
hj  a  board  or  large  sand-bag  against  \\hich  the  feet  can  rest. 

An  outline  is  given  elsewhere  of  the  forms  of  local  inflammation  of 
these  nerves,  with  special  reference  to  the  morbid  process,  on  account 
of  its  important  practical  relations.     (See  Crural  Neuritis,  p.  133.) 


BRACHIAL  NEURITIS. 


Besides  the  forms  of  inflammation  of  the  nerves  of  the  arm  already 
described,  a  primary  inflammation  of  the  brachial  plexus  occurs  in  a 
form  as  well  defined  as  sciatica,  and  equally  meriting  a  separate 
description.  This,  however,  need  only  include  its  special  features; 
those  that  are  common  to  other  forms  of  neuritis  have  been  already 
described.  It  is  closely  analogous  to  sciatica,  and  is  usually  a  peri- 
neuritis,— a  primary  inflammation  of  the  sheaths  of  the  branches  that 
enter  and  form  the  brachial  plexus. 

Cases  occur,  however,  in  which  the  symptoms  correspond  in  general 
character  to  the  rest,  but  in  which  their  distribution  suggests  that  the 
n^rve-roots  rather  than  the  plexus  are  the  seat  of  the  inflammation, 
and  pain  about  the  spine  supports  the  opinion.  This  form  may  be 
called  Radicular  Neuritis.  Although  its  existence  has  not  been 
established  by  pathological  evidence,  the  symptoms  mentioned  admit 
of  no  other  explanation.  A  knowledge  of  their  significance  is  of  great 
practical  importance  on  account  of  the  closeness  with  which  they  may 
simulate  those  of  organic  disease  of  the  bones  of  the  spine,  or  a  growth 
in  the  spinal  membranes. 

Causes. — The  influence  of  gout  in  causing  local  neuritis  is  very 
conspicuous  in  the  brachial  form,  with  the  special  characteristic  that 
this  occurs  chiefly  late  in  life,  verv  often  from  the  inherited  disease, 


120  BRACHIAL  NEURITIS. 

and  with  greater  frequency  in  females  than  any  other  form  of  neuritis ; 
their  liability  is  at  least  equal  to,  if  not  greater  than,  that  of  men. 
Fiye  sixths  of  the  cases  occur  after  fifty,  and  it  may  be  met  with  up 
to  extreme  old  age.  In  men  there  have  usually  been  the  ordinary 
manifestations  of  gout,  but  in  women  the  tendency  is  often  only 
indicated  by  the  family  history,  and  by  previous  muscular  rheuma- 
tism, especially  lumbago  and  sciatica, — which  have  seldom  been  abront 
in  either  sex.  In  one  case  the  onset  of  the  brachial  neuritis  occurred 
immediately  after  an  attack  of  sciatica.  Neuritis  of  the  brachial 
plexus  may  also  arise  from  trauma  or  the  pressure  of  new  growths, 
e.g.  of  a  pressure  on  the  posterior  surface  of  the  clavicle. 

Symptoms. — Pain,  the  great  symptom  of  the  inflammation  of  all 
mixed  and  sensory  nerves,  is  greater  in  this  than  in  most  forms  of 
neuritis.  It  is  usually  the  first  symptom,  and  lasts  long  after  the 
inflammation  is  over;  its  severity,  coupled  with  the  age  of  most 
sufferers,  renders  the  malady  one  of  a  peculiarly  trying  character. 
The  first  pain  is  often  referred  to  a  distance  from  the  seat  of  the 
inflammation,  perhaps  because  this  begins  at  the  plexus,  where,  at 
divisions,  it  is  facilitated  by  motion,  and  the  conducting  fibres  are 
readily  reached  and  early  irritated.  Frequent  seats  for  the  first  pain 
are  the  region  of  the  scapula  (sometimes  beneath  the  bone)  and  the 
wrist  or  back  of  the  forearm,  with  or  without  the  hand.  In  other 
cases,  however,  the  first  pain  is  at  the  plexus  itself,  above  the  clavicle 
or  in  the  axiUa,  and  these  are  the  places  in  which  it  is  commonly  most 
intense  throughout  the  attack.  As  the  pain  increases  it  extends  along 
the  course  of  the  nerves  of  the  arm,  which  the  patient  will  often  accu- 
rately indicate  with  his  finger  when  tracing  the  lines  of  pain. 

The  pain  is  sometimes  sudden  in  onset  and  severe  from  the  begin- 
ning ;  more  often  it  is  at  first  occasional,  or  felt  on  certain  movements, 
but,  as  it  increases,  it  becomes  more  continuous,  with  variations  that 
soon  become  paroxysmal.  Ultimately  there  is  always  more  or  less  dull 
wearying  j)ain  in  the  whole  arm,  but  especially  in  the  region  of  the 
plexus,  varied  by  attacks  of  great  severity.  In  these  the  pain  is  acute 
and  lancinating,  or  stabbing,  or  burning  ;  it  usually  takes  the  course 
of  the  nerves,  diffusing  itself  from  them,  and  often  passing  to  the  side 
of  the  chest  and  to  the  neck, — seldom  to  the  head.  The  pain  often 
varies  in  character  according  to  intensity ;  at  the  height  of  the 
paroxysm  it  may  be  sharp  and  stabbing,  or  such  darting  pains  may 
be  superadded  to  a  more  diffused  burning  pain,  which  lasts  longer 
than  the  acute  pain,  and  may  be  followed,  as  the  paroxysm  subsides, 
by  general  tingling  of  the  skin  of  the  whole  limb.  The  paroxysms  are 
induced  by  movement  or  occur  spontaneously.  In  slighter  cases  the 
pain  is  paroxysmal  only,  and  then  the  relation  to  movement  is  a  very 
conspicuous  feature.  Although  it  is  seldom  confined  to  movement,  this 
never  fails  to  induce  severe  pain,  and  the  patient  avoids  the  slightest 
use  of  the  limb.     Elevation  of  the  arm  especially  causes  distress. 

With  the  pain  there  is  usually  undue  sensitiveness  of  the  skin. 


DIAGNOSIS.  121 

which  may  be  much  increased  during  and  after  the  paroxysms.  Loss 
of  sensation  is  rare,  and  is  met  with  only  in  cases  that  are  not  only 
severe  but  prolonged.    The  two  may  concur,  as  "  anaesthesia  dolorosa." 

The  muscles  usually  present  the  flabbiness  and  slight  wasting 
common  in  neuritis,  but  the  damage  to  the  motor  fibres  is  seldom 
sufficient  to  cause  considerable  atrophy.  Sometimes,  however,  there 
is  enough  damage  to  cause  wasting  of  some  group  of  muscles,  with 
the  reaction  of  degeneration.  This  is  most  frequent  in  the  radicular 
form,  in  which  the  damage  is  to  the  upper  part  of  the  plexus  and 
nerve-roots.  There  may  then  be  anaesthesia  of  the  shin  over  the 
affected  muscles.  It  is  difficult  to  ascertain  the  existen<'e  or  the 
amount  of  motor  weakness;  effort  induces  pain  so  readily  that  the 
patient  can  seldom  be  induced  to  make  an  attempt  to  exert  force. 
Power  is  often  said  to  be  almost  lost,  when  it  is  probably  greater  than 
is  believed.  Besides  the  muscles,  the  subcutaneous  tissue  of  the  limb 
may  also  waste,  and  the  skin  may  become  thin  and  shining,  and 
present  the  aspect  already  described.  Subcutaneous  oedema  is  also 
common,  and  in  one  case  erythema  nodosum  was  found.*  Arthritic 
changes  in  the  joints  of  the  fingers  are  almost  constant  in  the  cases 
that  occur  in  later  life ;  the  adhesions  may  be  permanent. 

Diagnosis. — Few  maladies,  as  a  fact  of  experience,  give  rise  to 
greater  diagnostic  difficulty.  This  is  due  to  several  causes :  the 
affection  is  rare  ;  its  symptoms  are  sometimes  equivocal ;  the  subjects 
are  usually  in  the  degenerative  period  of  life,  when  ma.ny  obscure 
diseases  attended  with  pain  in  the  arm  occur  to  the  mind  of  the 
physician ;  and  lastly,  the  distinction  between  neuralgia  and  neuritis 
is  often  difficult,  although  less  so  than  is  supposed.  The  last  is,  indeed, 
the  mosb  frequent  source  of  error.  The  most  severe  and  characteristic 
cases  of  brachial  neuritis  are  frequently  mistaken  for  pure  neuralgia, 
on  account  of  the  paroxysmal  character  of  the  more  severe  pain,  and 
because  the  characters  of  neuritis  are  unfamiliar.  The  points  to  deter- 
mine the  diagnosis  are  those  described  in  the  sections  on  neuritis  and 
neuralgia ; — the  degree  of  persistent  tenderness  of  the  nerves  and  the 
influence  of  movement,  together  with  the  history  of  the  attack,  the 
locality  of  the  pains,  and  especially  any  evidence  of  damage  to  the 
fibres.  A  history  of  neuralgia  in  the  person  or  the  family  is  of  slight 
weight  only ;  the  tendencies  to  true  neuralgia  and  to  neuritis  are  often 
combined,  and  each  is  a  frequent  result  of  gout.  A  far  greater  diffi- 
culty is  presented  by  the  cases  in  which  the  inflammation  remains 
slight  and  is  confined  to  the  plexus,  affecting  branches  where  the  con- 
ducting fibres  can  be  so  irritated  as  to  cause  distant  pain,  which  then 
becomes  the  leading  symptom.  This  is  often  paroxysmal,  and,  when 
on  the  left  side,  may  radiate  to  the  chest  and  be  associated  with  dis- 
turbed action  of  the  heart.  Angina  pectoris  is  often  thought  of  in 
such  cases ;  and  here  again  the  degenerative  age  may  increase  the 
difficulty  by  leading  to  the  presence  of  some  coincident  disease  of  the 
*  V.  Fraiikl  Hoihvvart,  ♦  Wien.  klin.  Wocbeuschrift,'  1897,  No.  1. 


122  BEACH  I AL  NEURITIS. 

heai-t,  or  by  making  true  angina  not  unlikely.  The  distinction  afforded 
by  the  nerve-tenderness  is  tlien  of  great  importance,  because  the  dis- 
propoi-tionate  amount  of  tenderness  (compared  with  the  amount  of 
pain)  is  more  emphatic  in  slight  than  in  severe  cases.  Persistent 
tenderness  with  only  paroxysmal  pain  should  always  suggest  neuritis. 
When  the  position  of  the  pain  is  cai-ef  uUy  examined,  its  relation  to 
the  plexus  and  branches  is  often  clnar.  It  is  important  to  remember 
that  all  nerve-pains  in  the  brachial  region  on  the  left  side  have  a 
tendency  to  resemble  anginal  pain  in  distribution,  and  to  be  associated 
with  cardiac  distress.  Probably  there  is  some  peculiar  tendency  for 
pains  in  this  part  to  disturb  the  action  of  the  heart;  a  common  physio- 
logical relation  may  underlie  both  the  nerve-pains  of  cardiac  angina 
and  the  cardiac  symptoms  of  nerve-pains.  Hence  this  secondary  dis- 
turbance does  not  neutralise  the  significance  of  the  special  signs  of 
neuritis.  In  some  cases  of  the  slighter  class,  the  pains  suggest  the 
idea  of  an  aneurism  ;  in  many  cases  of  brachial  neuritis  this  diagnosis 
has  been  made,  and  the  patient  has  had  to  endure  months  of  mental 
distress,  for  which  no  real  cause  was  in  existence.  Such  a  suspicion, 
in  the  absence  of  special  signs,  such  as  pressure- symptoms,  should 
only  be  entertained  if  the  pains  are  persistently  and  increasingly 
severe,  and  unaccompanied  by  any  considerable  amount  of  tenderness. 
In  all  these  cases,  moreover,  the  presence  of  the  gouty  diathesis  may 
be  allowed  weight  in  the  diagnosis. 

The  muscular  wasting,  slight  in  degree,  added  to  the  arthritic 
changes,  gives  rise  to  a  condition  which  may  be  mistaken  for  a  primary 
joint  affection  with  secondary  "  arthritic  atrophy  "  of  the  muscles.  It 
is  only  in  the  chronic  stage  that  this  error  is  possible.  A  careful 
attention  to  the  history  of  the  case  will  show  its  real  nature,  but  with- 
out this  attention  the  mistake  is  easy,  and  it  is  often  made. 

Prognosis.  — Except  in  its  most  trifling  degree,  brachial  neuritis  is 
a  tedious  malady ;  the  duration  of  every  severe  case  is  to  be  measured 
by  months,  and  often  more  than  a  year  elapses  before  the  patient  is 
free  from  pain.  Post-neuritic  pain  is  always  more  prolonged  in  the 
old  than  in  the  young,  and  the  age  of  the  subjects,  together  with  the 
amount  of  damage  to  the  nerves,  causes  suffering  to  last  longer  than 
in  almost  any  other  form.  To  these  causes  also  must  be  added  the 
degree  of  sensitiveness  of  the  affected  nerves,  exceeded  only  by  that  of 
the  fifth  nerve,  and  also  the  mobility  of  the  parts  in  which  the  plexus 
lies ;  this  involves  a  continued  cause  of  irritation,  brought  into  play 
as  soon  as  the  diminution  in  the  severer  pain  permits  the  patient  to 
employ  the  long  useless  arm.  Eelapses,  moreover,  are  not  uncommon. 
Recurrence  may  take  place  after  moderate  attacks,  but  seldom  occurs 
after  those  of  extreme  severity. 

Eiccovery  from  the  consequences  of  the  inflammation  is  not  always 
complete.  The  limb  often  remains  smaller  and  feebler,  with  a  liability 
to  tiugHng  and  to  cramp,  and  also  to  neuralgic  pains  under  the  in- 
fluence of  changes  in  the  weather,  &c.     But  the  most  serious  conse- 


SCIATICA.  123 

quences  are  the  alterations  in  the  ^jints.  These  are  very  frequent : 
the  fixation  occasioned  by  the  pain  permits,  and  the  tendency  to 
perverted  nutrition  produces,  adhesion  between  the  ai^ticular  surfaces, 
in  consequence  of  which  the  movement  becomes  permanently  re- 
strained. It  is  probable,  moreover,  that  the  constitutional  state  which 
imderlies  the  primary  malady  often  increases  the  degree  of  these 
joint-alterations.  The  shoulder,  wrist,  and  fingers  are  the  joints  that 
are  most  frequently  thus  rendered  stiff.  The  interference  with  the 
movement  of  the  fingers  is  especially  troublesome ;  the  joints  become 
painful  in  consequence  of  the  unavoidable  strain  on  the  tissues  when 
an  attempt  at  use  is  made. 

Treatment. — The  treatment  that  has  been  described,  as  needed  in 
neuritis  generally,  is  suitable  also  for  this  form,  and  its  details  need 
not  here  be  repeated.  Abstinence  from  movement  is  of  great  import- 
ance ;  the  occurrence  of  stiffness  of  the  joints  in  consequence  of  the 
immobility  of  the  limb  must  be  risked.  It  is  doubtful,  indeed,  whether 
the  tendency  to  this  is  much  increased  thereby,  for  the  effect  of  pain, 
in  preventing  sufficient  movement  to  maintain  the  normal  state  of  the 
joints,  is  not  materially  increased  by  rest  otherwise  enforced ;  whUe 
the  difference  between  a  little  movement  and  none,  during  the  acute 
stage  of  the  affection,  is  enough  to  make  a  considerable  difference  in 
the  effect  on  the  inflammation  of  the  nerves.  The  influence  of  cocaine, 
injected  under  the  skin,  is  very  beneficial,  but  it  is  needed  frequently 
in  severe  cases,  and  two  injections  (each  -^ — |-  gr.)  may  be  given 
daily  during  the  height  of  the  disease.  It  is  important  that  the  nerves 
should  not  be  irritated  by  massage  in  the  state  of  active  inflammation ; 
indeed,  no  therapeutic  measure  which  occasions  prin  can  do  as  much 
good  as  harm.  After  the  tenderness  has  subsided,  gentle  rubbing, 
such  as  is  agreeable,  may  be  employed  with  advantage.  Electricity  is 
only  needed  for  muscles  that  present  the  reaction  of  degeneration,  or 
to  aid  in  removing  after-pains.  In  one  severe  case,  associated  with 
multiple  neuromata  of  the  skin,  relief  resulted  from  excision  of  the 
outer  and  inner  cords  of  the  plexus.  The  excised  portions  showed 
some  interstitial  neuritis.* 


SCIATICA. 


The  most  frequent  of  all  forms  of  inflammation  of  a  single  nerve, 
unilateral,  and  primarily  of  the  connective-tissue  elements,  and 
especially  of  the  sheath,  is  that  of  the  sciatic.  The  fact,  coupled 
with  the  prominent  character  of  the  painful  and  disabling  symptoms, 
have  raised  "sciatica"  into  a  conspicuous  position  among  diseases  of 

*  Mauiy  and  Jiihiiiig,  '  Amer.  Journ.  of  Med.  Sci.,'  1874;  a  similar  case  of  septic 
origin  recorded  by  S.uids  and  Seguin  was  not  cured  by  excision  of  the  plexus. 


124  SCIATICA. 

the  nerves,  and  indeed  among  diseases  generally.  This  renders  desirable 
a  specially  full  description  of  the  malady,  fuller  than  strictly  corre- 
sponds with  its  subordinate  position  as  a  variety  of  neuritis. 

As  the  word  "  sciatica  "  is  commonly  used,  it  is  a  general  designation 
for  all  affections  of  which  the  chief  symptom  is  pain  in  the  region  of 
the  sciatic  nerve.  In  a  stricter  use  of  the  word,  however,  it  is 
applied  to  painful  affections  of  the  nerve  not  due  to  any  morbid  pro- 
cess outside  it ;  thus  limited,  it  practically  corresponds  to  inflamma- 
tion of  the  nerve.  Two  varieties  may  be  distinguished,  however, 
according  as  the  process  in  the  nerve  is  excited  by  primary  disease  in 
its  vicinity,  "  secondary  "  sciatica ;  and  "  primary  "  sciatica,  when  the 
pain  is  the  expression  of  disease  beginning  in  and  related  to  the  nerve 
itself.  Primary  sciatica  is  sometimes  regarded  as  a  neuralgia.  This 
view  is  in  the  main  erroneous ;  the  vast  majority  of  cases  of  sciatica 
are  really  cases  of  true  neuritis. 

Causes. — Sciatica  is  far  more  frequent  in  males  than  in  females ; 
the  proportion  has  been  very  variously  estimated.  The  best  evidence  of 
the  incidence  of  the  disease  is  that  presented  by  the  patients  treated  at 
the  Devonshire  Hospital,  Buxton,  of  which  an  analysis  of  1000  cases 
has  been  made  by  Dr.  E.  V.  Gibson.*  The  percentage  of  males  is  88'4, 
of  females  ll'6,t  giving  a  ratio  of  5  to  1,  which  is  probably  very  near 
the  truth.  The  disease  is  unknown  in  childhood,  and  rare  in  the  second 
decade  of  life.  It  does  not,  indeed,  occur  under  fifteen ;  in  more  than 
half  the  cases  it  begins  between  thirty  and  fifty,  and  between  fifty  and 
seventy  in  a  quarter.  Having  regard  to  the  smaller  number  of  persons 
living,  the  frequency  of  its  occurrence  must  be  regarded  as  at  leadt  as 
great  in  late  as  in  middle  life. J 

No  special  influence  of  sex  is  distinguishable  in  the  decade  forty  to 
fifty  :  the  proportion  of  males  to  females  is  that  of  the  whole  series. 
Under  forty  a  smaller,  and  over  fifty  a  larger  proportion  of  females 
suffer.  Exposure  to  the  exciting  causes  is  probably  more  common 
in  males  during  middle  life,  when  also  the  malady  is  promoted  by 
the  various  accelerating  influences  which  aid  the  active  development 
of  its  chief  constitutional  cause— gout.  But  it  must  not  be  forgotten 
how  comparatively  small  is  the  actual  number  of  cases  in  women. 

Underlying  most  cases  of  sciatica  is  either  the  state  of  definite  gout, 
or  that  rheumatic  diathesis  in  which  the  fibrous  tissues  suffer,  especially 
those  that  are  connected  with  the  muscles,  a  form  closely  connected  with 
common  gout  by  co-existence  or  descent.  It  occurs  frequently  in  those 
who  are  themselves  gouty,  who  present  the  personal  characteristics  of  the 
disease,  and  have  fostered  it  by  mode  of  life.     It  occurs  also  in  those  of 

*  Dr.  E.  Valentine  Gibson,  '  Lancet,'  1893,  vol.  i,  p.  860. 

t  884  femiiles,  116  males,  in  the  1000. 

X  The  percentage  distribution  of  the  1000  cases  at  Bnxtoii  is  as  follows: — The 
age  is  taken  at  which  the  first  attack  commenced.  Between  15  and  20,  14  eases; 
21  to  30,  159  cases ;  31  to  40,  310  cases ;  41  to  50,  248  cases  (one  quarter  of  the 
whole) ;  51  to  60,  187  cases ;  61  to  70,  71  cases  ;  71  to  80,  11  cases. 


CAUSES.  125 

gouty  inheritance,  but  who  themselves  have  been  abstemious,  and 
sometimes  present  a  weakly  constitution — thin,  pallid,  neurotic.  The 
latter  have  often  suffered  from  acute  articular  rheumatism  in  earlier 
life.  It  is  among  them  that  the  most  severe  cases  are  met  with,  in 
which  the  inflammation  spreads  to  other  nerves,  or  involves  the  sheath 
of  the  sciatic  with  other  structures,  and  that  it  develops  early  in 
life,  during,  for  instance,  the  second  fifteen  years.  This  constitutional 
state,  with  all  its  effects,  is  sometimes  met  with  when  there  is  only 
trifling  evidence  of  inheritance.  Some  unknown  influence  seems  to 
determine  its  development  in  intense  degree  as  a  congenital  tendency, 
manifested  by  rheumatic  troubles,  various  and  severe,  early  in  adult 
life — the  sporadic  occurrence  of  that  which  is  usually  inherited. 
There  is  no  evidence  of  a  direct  causal  relation  to  other  consti- 
tutional diseases.  Syphilis  has  been  supposed  in  some  cases  to  give 
rise  to  it,  but  the  cases  are  rare,  and  the  common  cause  can  seldom 
be  excluded. 

An  exciting  cause  is  to  be  traced  in  many  of  the  cases.  Exposure 
to  cold  is  the  most  frequent.  It  is  usually  local  exposure,  as  by  wet 
boots,  standing  in  water,  &c. ;  sometimes,  however,  a  general  chiU  of 
the  body  determines  an  attack.  The  exposure  to  cold  may  be  even 
more  direct,  as  by  sitting  on  wet  grass.  Draughty  water-closet  seats 
are  answerable  for  some  cases.  The  neuritis  often  arises  by  the  exten- 
sion of  an  adjacent  rheumatic  affection  of  the  fibrous  tissue,  especially 
of  that  form  of  "  lumbago  "  which  involves  the  fibrous  attachments  of 
the  muscles  at  the  back  of  the  sacrum,  less  commonly  in  its  ordinary 
lumbar  seat.  This  affection  passes  down  from  the  sacrum,  extending 
along  the  fasciae,  to  the  nerve- sheath  in  the  neighbourhood  of  the 
sciatic  notch ;  or  passes  forwards,  over  the  crest,  to  the  front  of  the 
iliac  bone,  and.  spreads  in  the  tissue  about  the  lumbar  plexus,  and 
descends  to  that  which  covers  the  mass  of  sacral  nerves  from  which 
the  sciatic  proceeds.  (The  fact  is  of  much  interest,  because  it  shows 
that  this  form  of  fibrous  rheumatism,  of  which  we  have  only  a  very 
vague  pathological  conception,  must  be  regarded  as  inflammation, 
since  positive  neuritis  results  from  it.)  The  rhetunatic  pain  has  usually 
existed  for  a  few  days  only  before  the  extension  occurs,  but  sometimes 
a  chronic  affection,  after  existing  as  such  for  several  weeks,  spreads 
acutely. 

Mechanical  disturbance  sometimes  excites  the  disease,  and  often 
co-operates  with  other  influences.  The  pressure  of  the  edge  of 
the  chair,  in  those  who  sit  much,  is  the  most  frequent.  Muscular 
over-exertion,  suddenly  compressing  the  nerve  in  the  thigh,  is  occa- 
sionally effective.  If  the  nerve  is  already  tender,  a  strong  contraction 
of  the  muscles  at  the  back  of  the  thigh,  especially  when  the  knee  is 
flexed,  and  the  muscles  can  freely  shorten  and  widen,  may  produce  acute 
pain  ia  the  nerve,  evidently  by  its  compression.  This  cause  is  probably 
effective  only  in  a  predisposed  person,  or  when  there  is  already  com- 
mencing neuritis 


126  SCIATICA. 

Varions  morbid  processes  within  the  pelvis  may  cause  sciatica  by 
compressing  the  sacral  plexus,  or  by  exciting  inflammation  which 
invades  the  nerve.  Rectal  and  other  tumours  give  rise  to  progressive 
pressure,  and  the  inflammation  excited  descends  the  nerve,  resembling 
the  primary  form.  It  may  be  an  early  symptom  of  a  growth  springing 
from  the  bone,  as  an  enchondroma  arising  at  the  sacro-iliac  synchon- 
drosis. Pelvic  inflammation  and  injury  during  labour  are  occasional 
causes.  A  loaded  rectimi  may  be  the  excitant,  but  is  a  rare  cause, 
although  apt  to  be  recurrent  when  once  effective.  Lastly,  the  sciatic 
nerve  may  be  secondarily  involved  in  mischief  that  is  outside  the 
pelvis.  The  most  frequent  cause  of  this  is  disease  of  the  bone,  as 
disease  of  the  hip- joint,  especially  senile  rheumatic  inflammation 
(Gribson). 

The  proclivity  of  the  fibrous  tissue  of  this  nerve  to  suffer  primarily 
is  due  to  its  position  and  the  exposure  this  involves,  and  to  its  con- 
nections, which  facilitate  the  passage  of  inflammation  to  it.  But 
there  are  cases  with  a  strong  disposition  for  fibrous  rheumatism  to  fix 
itself  in  the  tissues  of  the  pelvis,  sacral  and  lumbar  regions;  the 
nerves  cannot  escape  implication ;  whether  the  sciatic  suffers  early  or 
late,  and  in  what  degree,  depend  on  secondary  conditions,  but  it  is 
specially  liable  on  account  of  the  anatomical  relations  of  its  origin. 
The  mass  of  the  "  sacral  plexus  "  is  prolonged  into  it,  and  the  mem- 
branes covering  this,  and  its  branches,  including  the  lumbo-sacral  cord, 
are  very  liable  to  be  the  seat  of  such  inflammation,  wide- spread,  fixing 
itself  irregularly  on  the  various  nerves,  but  in  special  degree  on  the 
sciatic.  In  these  cases  the  pain  is  extensive,  and  often  severe  in  the 
front  of  the  thigh,  but  the  symptoms  of  descending  neuritis  are 
prominent  chiefly  in  the  sciatic. 

Symptoms.— The  two  nerves  suffer  with  nearly  equal  frequency,  but 
Gibson  found,  in  his  extensive  series,  that  the  left  was  a  little  the 
more  liable,  in  the  proportion  of  48  to  44  per  cent.  In  about  7  per  cent. 
both  were  affected  simultaneously — a  striking  indication  of  the  smaller 
relative  part  played  by  the  general  blood-state,  compared  with  poly- 
neuritis, and  the  preponderant  influence  of  local  excitants.  The 
chief  symptom  of  primary  sciatica  is  pain  along  the  course  of  the 
nerve-trunk,  often  also  along  that  of  its  branches;  pain  in  tlie 
area  of  its  distribution  is  sometimes  subsequently  developed.  The 
affection  may  begin  suddenly,  especially  in  cases  of  rheumatic  origin, 
—as  suddenly  as  lumbago.  Some  movement  seems  to  excite  it, 
but  there  has  usually  been  slighter  rheumatic  pain  in  the  neighbour- 
hood for  a  day  or  two,  generally  about  the  hip  or  sacrum.  More 
frequently  the  onset  is  gradual;  slight  pain  is  felt  along  the  back 
of  the  thigh,  on  movements  and  in  postures  that  make  the  nerve  tense, 
or  cause  pressure  upon  it.  This  pain,  due  to  a  slight  degree  of 
inflammation,  has  generally  existed  for  some  weeks,  increasing  in 
degree  until  a  considerable  severity  is  attained,  or  suddenly  becoming 
intense  under  the  influence  of  some  exposure  or  over-exertion.     At 


SYMPTOMS.  127 

last  the  patient  is  easy  only  when  at  rest,  and  when  the  leg  is  in  a 
certain  posture.  Any  movement  that  makes  the  nerve  tense  causes 
pain,  and  to  avoid  this  the  knee,  in  walking,  is  kept  slightly  flexed, 
and  the  leg  held  stiffly  so  as  to  avoid  stretching  the  nerve.  As  the 
pain  on  movement  increases,  spontaneous  pain  is  added,  at  first  chiefly 
felt  in  the  nerve-trunk,  but  soon  spreading  to  its  branches  and  distri- 
bution. It  is  usually  most  intense  in  certain  spots — (1)  above  the 
hip-joint,  near  the  posterior  iliac  spine,  (2)  at  the  sciatic  notch,  (3) 
about  the  middle  of  the  thigh,  (4)  behind  the  knee,  (5)  below  the 
head  of  the  fibula,  (6)  behind  the  external  malleolus,  (7)  on  the  back 
of  the  foot.  The  pain  may  radiate  over  the  whole  distribution  of  the 
nerve,  but  it  is  often  so  distinctly  limited  to  the  course  of  the  trunk 
and  branches,  that  the  patient  points  these  out  with  exactness  when 
he  indicates  its  course.  The  chief  intensity  of  the  pain  is  usually 
down  the  back  of  the  thigh  It  may  be  dull  or  acute,  is  often  burnmg 
in  character,  and  worse  at  night.  It  may  seem  to  dart  downwards, 
starting  from  the  highest  point.  As  the  pain  on  movement  increases, 
the  nerve-trunk  becomes  extremely  tender  to  pressure.  Even  Defore 
the  tenderness  becomes  considerable  in  the  thigh,  pain  may  often  be 
produced  in  the  following  manner : — Let  the  patient  sit  on  a  chair 
with  the  knee  at  a  little  more  than  a  right  angle,  and  the  body  bent 
forward,  so  as  to  lengthen  the  course  of  the  nerve  at  tlie  hip-  and 
knee-joints.  If  the  finger  is  then  pressed  into  the  popliteal  space,  so 
as  to  make  the  nerve  a  little  more  tense,  a  pain  is  felt  in  the  course  of 
the  nerve  at  the  back  of  the  thigh  or  above  the  sciatic  notch,  and  behind 
the  hip.  It  is  due  to  the  sensitiveness  of  the  nerve  to  tension,  and  is 
a  very  useful  test,  especially  when  the  part  inflamed  is  high  uj)  within 
the  pelvis.  It  may  reveal  the  affection  of  the  nerve  here,  by  making 
it  more  tense,  when  there  is  no  tenderness  to  pressure  at  the  back  of 
the  thigh. 

Abnormal  sensations  other  than  pain  are  often  felt  in  the  area  of 
distribution  of  the  nerve, — tingling,  formication,  and  the  like ;  and  in 
severe  cases  there  may  be  diminished  sensibility  on  the  back  of  the 
thigh,  on  the  leg,  or  the  foot.  The  affection  of  sensibility  at  the  back 
of  the  thigh  indicates  that  the  disease  extends  up  the  nerve,  above  the 
sciatic  notch,  to  the  origin  of  the  small  sciatic,  or  that  this  is  involved 
in  a  simultaneous  neuritis.  In  severe  cases  the  muscles  supplied  by 
the  nerve  become  flabby,  tender  to  the  touch,  and  sometimes  distinctly 
weak  and  wasted.  This  is  chiefly  noticeable  in  the  calf  muscles, 
and  in  the  group  supplied  by  the  external  popliteal  nerve.  A 
tendency  to  cramp  in  the  muscles  is  often  very  marked.  There 
may  be  an  alteration  in  the  electrical  irritability,  usually  a  sHghl; 
increase  to  each  form ;  it  is  considerable,  and  amounts  to  a  distinct 
degenerative  reaction,  only  in  very  severe  cases.  SKght  fever  and 
corresponding  constitutional  symptoms  may  attend  an  onset  that  is 
acute,  when  the  inflammation  is  intense.  Chronic  cases,  however, 
are  usually  not  attended  by  elevation  of  temperature. 


128  SCIATICA. 

The  duration  and  severity  of  tlie  affection  are  extremely  rariable. 
They  depend  on  its  intensity,  and  on  tlie  amount  of  rest  given  to  the 
limb  in  the  early  stage,  and  on  the  constitutional  state  of  the  patient. 
The  inflammation  may  be  trifling  in  degree,  causing  pain  on  move- 
ment only,  which  may  pass  away  in  the  course  of  a  few  weeks.  On 
the  other  hand,  the  spontaneous  pain  may  be  so  continuous  and 
intense  that  sleep  can  be  obtained  only  by  the  help  of  narcotics,  and 
the  disease  may  continue  for  many  months,  and  even  for  a  year.  In  most 
cases  that  last  more  than  a  year  there  is  partial  recovery  and  relapse. 
Improvement  is  shown  first  by  the  subsidence  of  spontaneous  pain, 
followed  by  the  slow  diminution  of  the  pain  on  movement,  and  then 
of  the  tenderness  of  the  nerve.  The  muscular  wasting,  which  occurs 
in  severe  cases,  may  last  long  after  the  active  stage  is  over  ;  fibrillary 
contractions  in  the  muscles  that  have  been  affected  may  continue 
for  years,  and  are  often  accompanied  by  a  strong  tendency  to  cramp, 
which  may  be  excited  by  voluntary  contraction.  Occasionally  a  secon- 
dary neuralgia  is  set  up,  which  may  be  very  enduring,  may  involve 
the  entire  length  of  the  nerve,  and  may  be  wide  in  distribution,  ex- 
tending outside  the  sciatic  area. 

The  disease  is  prone  to  relapse,  and  still  more  prone  to  recur  after 
recovery.  A  second  attack  may  occur  in  the  same  or  in  the  other 
leg,  but  both  legs  are  scarcely  ever  affected  at  the  same  time.  At 
last,  however,  the  tendency  of  the  sheath  to  be  inflamed  seems  to 
become  exhausted,  and  the  liabitity  to  cease. 

The  cases  of  secondary  sciitica  depending  on  disease  outside  the 
nerve,  compressing  or  irritating  it,  differ,  in  some  respects,  from  the 
primary  form.  The  early  pain  is  felt  less  in  the  nerve-trunk  than  in 
its  distribution,  especially  when  the  nerve  suffers  first  by  pressure; 
interference  with  the  conducting  functions  is  more  conspicuous  in  the 
early  stage.  The  primary  form  may  be  afterwards  closely  simulated, 
because  secondary  inflammation  may  descend  the  nerve  and  induce 
the  same  tenderness  of  the  nerve-trunk.  The  course  of  the  secondary 
cases  is  more  progressive,  but  it  depends  on  that  of  the  original 
disease. 

Complications  of  sciatica  are  rare,  but  one  or  two  deserve  mention. 
Cutaneous  eruptions,  usually  herpetic  in  character,  have  been  occa- 
sionally met  with.  These  may  heal  slowly,  but,  in  themselves,  they 
are  unimportant.  Sometimes  the  epidermis  exfoliates ;  oedema  of  the 
limb  occurs  in  rare  cases.  These  are,  however,  rather  effects  of  the 
neuritis  than  complications,  and  so  also  is  the  muscular  wasting  that 
is  occasionally  pronounced.  The  trophic  changes  in  the  skin  are  the 
expression  of  a  peculiarly  irritative  character  of  the  inflammation. 

Occasionally  the  symptoms  of  inflammation  of  one  sciatic  nerve  are 
attended  by  bilateral  symptoms,  especially  at  the  onset,  such  as 
tingling  or  pains  in  the  feet  and  the  soles,  or  some  weakness  of  the  flexor 
muscles  of  both  ankles.  These  indicate  an  associated  poison ;  it  may 
be  alcohol  causiug  its  own  peculiar  bilateral  peripheral  neuritis,  and 


PATHOLOGY.  129 

aiding  in  the  generation  of  their  acute  gouty  effects  on  the  sciatic. 
Such  a  combination  may  puzzle  the  observer. 

A  rare  complication  is  a  tendency  of  the  mischief  to  ascend  the 
nerve.  Thus  symptoms  may  spread  from  the  region  of  the  sciatic 
to  that  of  the  lumbar  plexus  ;  the  pain  may  spread  to  the  front  of 
the  thigh,  and  the  extensors  of  the  knee  may  become  flabby  and 
weak.  This  is  probably  due  to  the  passage  of  an  ascending  neuritis 
up  the  lumbo-sacral  cord.  In  extremely  rare  cases  the  morbid  pro- 
cess has  apparently  reached  the  spinal  cord,  and  indications  of  cord 
disease  (e.  g.  disordered  sensibility  in  the  soles)  have  thus  succeeded 
those  of  a  primary  and  apparently  simple  sciatica.  There  may  even  be 
symptoms  of  lateral  sclerosis  (excessive  knee-jerk,  rectus  clonus,  &c.), 
such  as  develop  in  some  cases  of  arthritis  and  other  conditions  of  pro- 
longed pain. 

The  wider  distribution  of  the  pain  and  other  symptoms,  when  a 
rheumatic  inflammation  is  extensive  from  the  first,  may  have  the 
aspect  of  a  complication,  but  often  the  involvement  of  the  sciatic  does 
not  dominate  the  case,  which  is  then  rather  one  of  general  crural 
neuritis,  the  result  of  the  affection  of  the  fibrous  tissue  of  the  floor 
of  the  pelvis  where  numerous  nerves  arise.  The  position  of  some  of 
them  determines  the  frequency  with  which  they  suffer  specially,  but 
the  size  of  the  sciatic  nerve-mass  necessarily  causes  its  affection 
to  be  chiefly  conspicuous.  That  of  some  of  the  lowest  branches  of 
the  lumbar  plexus  also  determines  the  common  seats  of  the  effects 
of  extension  upwards. 

In  spreading  cases,  one  effect  of  extension  is  of  particular  import- 
ince — the  paralysis  of  the  bladder  that  may  result  from  extension 
to  its  nerves.  The  nearer  part  of  the  lumbar  plexus  is  often  then 
involved,  and  pain  is  felt  ia  the  front  and  outer  part  of  the  thigh,  and 
even  in  the  groin  and  lower  part  of  the  abdomen.  The  vesical  nerves 
from  the  pelvic  plexuses  of  the  two  sides  are  near  together,  and  the  palsy 
of  the  bladder  is  often  complete,  and  of  long  duration,  outlasting,  it  may 
be,  all  other  symptoms.     Fortunately,  it  is  a  very  rare  complication. 

Pathology.  —  The  evidence  of  pathological  changes  is  scanty. 
Rheumatic  inflammation  of  the  nerve  is  scarcely  ever  met  with  in  its 
active  stage ;  the  disease  is  not  one  which  is  even  attended  by  death 
from  other  causes,  and  the  morbid  changes  are  a  matter  of  inference. 
There  is,  indeed,  hardly  any  organic  disease  so  common  as  sciatica,  of 
which,  in  its  various  degrees,  opportunities  for  pathological  observa- 
tion are  so  few  and  so  meagre.  Signs  of  inflammation  have  been 
found,  chiefly  conspicuous  in  the  sheath  of  the  nerve,  and  invading 
the  interstitial  tissue  in  more  severe  and  prolonged  cases.  They 
are  such  as  are  met  with  in  all  forms  of  perineuritis — swelling  and 
redness,  most  distinct  opposite  the  middle  of  the  thigh — thickening 
of  the  sheath,  invading  the  substance  of  the  nerve.  Microscopical 
changes  have  been  found,  similar  to  those  in  acute  neuritis  elsewhere^ 
in  the  rare  opportunities  for  observation  that  have  been  met  with. 
VOL.  I.  9 


I'SU  SCIATICA. 

The  existence  of  neuritis  in  all  severe  cases  admits  of  no  doubt; 
tlie  symptoms — wasting  of  tlie  muscles  and  anaesthesia — indicate 
structural  damage  to  the  nerve-fibres,  and  preclude  any  other  explana- 
tion. There  is  every  gradation  between  these  severe  forms  and  those 
which  are  slight,  and  the  symptoms  in  the  latter  are  identical  with 
those  of  the  earlier  stage  of  the  severe  cases.  The  early  local  tender- 
ness proves  the  initial  affection  of  the  sheath  and  fibrous  tissue.  All 
cases  of  definite  persistence  and  prolonged  course  are  inflammatory. 
Only  transient  pains  coming  and  going,  associated  "svith  neuralgia  else- 
where, can  be  regarded  as  of  neuralgic  character.  These  are  often 
the  sequel  to  a  true  neuritis,  or  are,  not  seldom,  the  manifestation  of 
a  tabetic  neuralgia,  the  pain  of  which  may  recur  during  years  in  a 
neuralgic  form,  and  even  be  influenced  in  its  incidence  by  change  in 
the  weather,  so  as  to  receive  a  rheumatic  character. 

The  symptoms  are  explained  by  the  morbid  process,  and  the  facts  of 
neuritis  in  general,  already  described,  apply  to  this  disease  in  every 
point.  It  is  primarily  a  perineuritis ;  the  pain  m  the  nerve  and  its 
tenderness  are  due  to  irritation  of  the  sheath-nerves.  The  pain  re- 
ferred to  the  distal  portions  of  the  nerve  is  due  to  the  irritation  of  its 
conducting  fibres  by  the  interstitial  inflammation,  while  their  greater 
damage  explains  the  sensory  impairment  and  muscular  wasting. 

The  disease,  like  other  forms  of  irregular  neuritis,  is  not  one 
essentially  of  the  nervous  system.  It  is  a  local  effect  of  the  con- 
stitutional malady,  gout,  or  the  fibro-rheumatism  which  results  from 
the  inherited  diathesis,  and  each  seems  to  depend  on  the  presence  of 
chemical  toxines  in  the  blood,  generated  in  the  body  by  abnormal 
metabolic  processes.  Uric  acid  is  probably  only  one  of  these,  and  not 
the  most  harmful,  or  that  which  has  the  most  special  influence.  But 
the  nature  and  origin  of  these  is  still  one  of  the  most  obscure  as  it 
is  one  of  the  most  important  problems  of  pathology,  and  we  must 
assume  that  it  acts  upon  tissues  disposed  to  degraded  nutrition  by 
the  influence  of  inherited  tendency. 

Diagnosis. — The  distinction  between  a  sciatic  neuritis  and  a  sciatic 
neuralgia  is  sometimes  difficult,  although  less  frequently  than  might  be 
inferred  from  current  accounts  of  these  diseases,  in  which  the  history 
of  the  neuralgia  has  been  written  from  the  symptoms  of  the  neuritis. 
If  we  recognise  that  all  cases  of  sciatica  with  persistent  tenderness  of 
the  nerve  are  really  neuritic,  cases  of  sciatic  neuralgia  become  extremely 
rare.  The  two  diseases  occur  usually  under  different  conditions  :  the 
subjects  of  neuralgia  have  often  suffered  from  neuralgia  elsewhere, 
.and  are  generally  weakly  and  anaemic.  The  pain  is  from  the  first 
spontaneous  ;  it  occurs  as  part  of  more  extensive  and  severe  attacks  ; 
posture  has  little  influence  upon  it ;  movement  is  not  itself  painful, 
although  it  may  excite  paroxysms  of  pain.  The  pain  is  referred  to  the 
branches  and  distribution  of  the  nerve  rather  than  to  its  trunk,  or  it 
■darts  up  or  down  the  trunk  ;  and  tenderness  of  the  nerve,  if  it  exists, 
is  altogether  subordinate  to  the  spontaneous  pain. 


TREATMENT.  131 

When  the  nerve  is  only  one  of  many  localities  in  which  the  pain  is 
felt,  and  the  patient  is  an  adult,  the  possibility  of  its  tabetic  nature 
must  be  remembered.  This  is  practically  certain  if  the  knee-jerks 
are  assuredly  absent,  but  of  this  fact  there  must  be  no  question.  The 
"  lightning  "  character  of  the  pain  is  an  important  diagnostic  feature 
in  such  cases ;  although  the  presence  of  this  character  has  almost 
pathog-nomonic  significance,  pain  of  less  acuteness  and  brevity  is 
often  significant.  It  is  an  instance  in  which  a  certain  feature  has 
great  significance,  but  its  absence  is  of  relatively  small  value. 

Secondary  sciatica  is  usually  produced  by  disease  of  bone  about  the 
hip-joint,  or  of  the  joint  itself,  or  by  disease  in  the  pelvis.  In  the 
former  case  a  careful  examination  (never  to  be  omitted  in  any  case  of 
sciatica)  at  once  reveals  the  mischief.  When  the  disease  is  within  the 
pelvis,  and  is  not  primarily  connected  with  the  nerves  (as  the  pressure 
of  a  growth),  the  tenderness  of  the  trunk  of  the  nerve  is  slight  in  pro- 
portion to  the  pain  felt  in  its  distribution ;  this  circumstance  should 
always  lead  to  a  careful  search  for  any  indication  of  pelvic  mischief. 
In  any  case  of  doubt,  or  of  prolonged  course  with  steady  increase  in 
the  symptoms,  a  rectal  examination  should  be  made. 

Sciatic  pain  occurs  in  some  diseases  of  the  bones  of  the  spine,  in 
lesions  of  the  cauda  equina,  and  occasionally  in  disease  of  the  spinal 
cord  itself.  In  these  cases  we  have  little  or  no  tenderness  of  the 
nerve ;  the  pain  is  chiefly  peripheral,  and  very  often  bilateral.  Double 
true  sciatica  is  so  rare  that  bilateral  pain  should  always  suggest  disease 
of  the  nerve-roots ;  but  the  pain  in  these  cases  is  generally  irregular 
in  its  distribution,  and  considerable  in  other  nerve  areas  in  the  leg  or 
neighbourhood  of  the  hip,  and  is  often  increased  much  by  movement 
of  the  spine. 

These  cases  of  "secondary  sciatica"  are  of  much  practical  import- 
ance, because  their  nature  is  usually  mistaken ;  for  a  long  time  the 
patient  is  thought  to  be  the  subject  of  the  ordinary  more  common 
form,  and  is  treated  accordingly,  even  when  the  character  of  the  pain 
should  raise  a  suspicion  of  the  nature  of  the  case. 

Prognosis. — The  prognosis  of  sciatica,  not  secondary  to  disease 
outside  the  nerve,  is  good  as  regards  ultimate  recovery,  but  is  very- 
uncertain  as  regards  severity  and  duration.  As  a  general  rule,  these 
features  are  proportioned,  but  both  are  influenced  by  the  practicability 
of  adequate  rest.  Irritating  exertion  may  lengthen  the  duration  of  the 
disease  by  many  months,  and  indeed  relapse  may  follow  each  partial 
recovery  for  one  or  two  years.  Spontaneous  pain,  and  that  which  is 
referred  to  the  distribution  of  the  nerves,  make  the  prospect  less 
favourable. 

Treatment. — The  principles  of  the  treatment  of  sciatica  are  those 
of  neuritis  already  described,  and  only  the  points  of  special  character 
need  be  here  repeated.  In  all  crises  rest  to  the  limb  is  important  beyond 
any  other  measure,  and  its  urgency  is  proportioned  to  the  acuteness 
a,nd  severity  of  the  symptoms.     Many  slight  cases  are  converted  into 


132  SCIATICA. 

serere  ones  by  unwise  exertion.  Every  posture  that  induces  pain,  and 
all  movements  which  increase  that  which  exists,  should  be  avoided. 
The  same  is  true  of  pressure  on  the  nerve  by  a  hard  seat,  and  by  strong 
contraction  of  the  flexors  cf  the  knee.  Pain  may  be  thus  suddenly 
brought  on  in  severe  degree,  and  may  endure. 

In  more  positive  treatment  the  causes  of  the  disease  must  be 
remembered,  and,  among  these,  the  frequency  with  which  it  is  related 
to  a  gouty  or  rheumatic  blood-state.  In  gouty  cases  saline  purgatives 
are  often  of  signal  service,  and  are  distinctly  usefiil  in  preventing 
attacks  in  those  who  are  liable.  In  the  acute  stage  of  a  severe  attack, 
hot  linseed-meal  poultices  should  be  applied  along  the  course  of  the 
nerve.  Counter-irritation  is  of  great  value,  and  cannot  be  employed 
too  early.  A  commencing  attack  may  often  be  cut  short  in  a  few  days 
by  rest,  and  a  series  of  mustard  plasters  or  smaU  blisters  appHed  over 
the  seats  of  pain,  moved  as  this  changes  under  their  influence,  chasing 
it,  as  it  were,  from  one  spot  to  another,  until  it  disappears.  Internally, 
whenever  there  is  reason  to  believe  that  active  inflammation  exists, 
mercury  should  be  given — a  grain  of  blue  pill  twice  daily ;  nothing 
else  seems  distinctly  to  influence  the  process.  Salicylate  of  potash  or 
lithia,  and  nitrous  ether,  should  also  be  given  at  the  onset — soda  salts 
being  avoided.  Spontaneous  pain  can  only  be  relieved  by  sedatives. 
Morphia  is  the  surest,  but  it  should  only  be  used  for  the  relief  of 
severe  spontaneous  pain.  Cocaine  often  proves  of  great  service,  if 
it  can  be  combined  with  rest,  and  injected  at  the  actual  source  of 
the  pain.  It  should  be  injected  pretty  deeply  at  this  place,  but 
not  into  the  nerve.  One  twelfth  of  a  grain  may  be  first  used, 
increased  rapidly  to  a  third  or  half  a  grain.  It  generally  relieves, 
although  not  in  the  same  degree  as  morphia,  but  it  seems  to  have 
more  influence  in  promoting  the  subsidence  of  the  inflammation,  as 
described  in  the  account  of  neuritis,  apparently  because  it  has  a  more 
distinct  influence  on  the  conduction  of  irritating  impulses  along  the 
nerves.  Morphia,  although  equally  effective  elsewhere,  may  also  be 
injected  with  advantage  over  the  inflamed  part  of  the  nerve,  so  as  to 
combine  the  counter- irritation  of  cutaneous  acupuncture  with  some 
local  as  well  as  general  sedative  influence.  Simple  acupuncture  along 
the  course  of  the  nerve  has  been  recommended:  it  gives  temporary 
relief  to  the  sciatic  pain,  as  to  any  superficial  pain,  but  the  cases  are 
verv  few  in  which  it  has  a  permanent  effect.  Sedative  or  counter- 
irritant  liniments  and  ointments  may  also  be  applied  along  the  course 
of  the  nerve ;  the  most  useful  are  belladonna  liniment  mixed  with  an 
equal  part  of  chloroform  liniment,  and  aconite  ointment,  rubbed  in 
until  distinct  tingling  is  produced.  Electricity  is  chiefly  useful  in  the 
later  stages ;  its  method  of  use  has  been  described  in  the  account  of 
the  treatment  of  neuritis.  In  very  obstinate  cases,  nerve-stretching 
has  done  good;  sometimes,  perhaps,  by  releasing  the  nerve  from 
compressing  adhesions,  but  probably  more  often  by  effecting  an 
energetic  counter- irritation,  and  enforcing  a  beneficial  rest.     It  has 


GENERAL   CRURAL  NEURITIS.  133 

ceased  to  be  tie  fasMon  it  was  a  few  years  ago,  proof  of  its  limited 
power.  Forced  movement  of  the  joints,  followed  loj  rest,  has  been 
sometimes  employed.  In  severe  cases  absolute  rest  in  bed,  and,  if 
necessary,  the  application  of  a  splint  to  ensure  this,  has  been  recom- 
mended. "Weir  Mitchell*  has  urged  the  use  of  a  long  splint,  reaching 
from  the  axilla,  jointed,  so  that  the  hip  and  knee-joints  may  be  slightly 
varied  in  position,  and  the  combination  with  this  of  a  soft  flannel 
bandage  to  the  limb.  The  latter  should  be  reapplied  twice  daily,  from 
the  foot  to  the  groin,  to  lessen  the  amount  of  blood  in  the  limb.  If 
further  measures  are  necessary,  the  application  of  the  Paquelin 
cautery  to  the  tender  points,  or  of  tin  splints  filled  with  ice  water, 
is  recommended,  and  after  recovery  massage  with  plentiful  feeding. 

To  prevent  recurrence  the  causes  of  gout  should  be  carefully 
avoided,  and  any  signs  of  a  gouty  state  lessened  by  an  appropriate 
regimen.  Habitual  saline  aperients  are  very  important ;  the  "  flush 
out  "  clears  away  bile  that  would  be  reabsorbed,  and  the  result  is  the 
same  as  increased  secretion  from  the  liver.  Slight  pain,  threatening  a 
recurrence,  should  be  met  by  more  rest,  by  counter-irritation  with  sma^ 
pisms,  and  the  careful  avoidance  of  sudden  movements,  and  indeed  ot 
all  exertion  that  causes  or  increases  pain.  If  this  is  considerable,  or 
the  nerve  is  distinctly  tender,  complete  rest  for  a  day  or  two  may 
obviate  the  subsequent  need  for  weeks  of  rest.  If  there  is  the  sacro- 
lumbago  that  often  precedes  sciatica,  the  pain  produced  by  sudden 
movement  may  often  be  lessened  by  a  few  moments'  rubbing. 


General  Crueal  TTeiteitis. 

■  In  rare  cases.  "  rheumatic "  inflammation  of  the  fibrous  tissue 
■covering  the  lumbar  and  sacral  plexuses  is  so  extensive  that  many  or 
most  of  the  nerves  to  the  leg  are  involved,  in  consequence  of  the 
affection  of  their  coverings  at  their  origin.  That  of  the  sciatic  may 
preponderate,  as  already  mentioned  in  the  section  on  Diagnosis, 
where  the  general  characters  of  this  form  are  referred  to,  and  its 
secondary  origin  by  extension,  in  some  cases  of  primary  sciatic  neuritis. 
The  cases  in  which  the  affection  of  the  fibrous  membranes  is  extensive 
give  rise  to  such  wide-spread  symptoms,  pain  taking  the  com-se  of 
many  nerves  and  accompanied  by  the  tenderness  of  descending 
neui-itis  and  impairment  of  movement,  often  chiefly  from  the  inhibitory 
influence  of  the  pain  it  causes,  attended  by  moderate  muscular  wasting. 
There  is  usually  slight  increase  or  decrease  in  the  electric  irritability 
in  some  group  of  muscles,  with  increased  knee-jerk,  very  rarely  the 
reaction  of  degeneration  where  the  nerves  have  suffered  severely,  and 
then  the  knee-jerk  may  be  lost  if  these  are  the  extensors  of  the  knee. 
In  cases  of  general  crural  neuritis  the  extent  of  the  symptoms  causes 
them  to  simulate  such  as  are  caused  by  disease  of  the  spinal  cord, 

*  Weir  Mitchell, '  International  Clinic,'  1891. 


134  NEURO-MYOSITIS. 

and  tlie  distinction  may  be  one  of  real  difficulty.  But  it  usually 
suffices  to  be  aware  of  its  occurrence  and  general  characters,  especially 
the  moderate  degree  of  the  objective  symptoms,  and  the  existence  of 
tenderness  of  sonie  nerve-trunks  near  the  pelvis.  The  diagnosis  is 
also  aided  by  the  fact  that  it  occurs  chiefly  in  those  who  present  a 
gouty  or  rheumatic  disposition,  acquired  or  inherited.  The  difficulty 
may  be  increased  by  the  occasional  presence  of  reflected  pain  on  the 
opposite  side,  distinguished,  however,  by  its  slighter  character,  and 
correspondence  in  seat  to  that  which  is  most  severe  on  the  side  chiefly 
affected.  The  course  of  these  cases  is  often  prolonged,  and  months 
may  pass  before  improvement  commences,  especially  when  the  under- 
lying constitutional  state  is  not  recognised,  the  treatment  of  which,  as 
in  sciatica,  is  of  primary  importance. 

Anterior  Crural  Neuritis. — Such  an  affection  of  the  fibrous  tissue 
may  be  liraited  to  that  related  to  a  single  nerve,  or  some  other  branch 
of  the  sacral  or  lumbar  plexus  may  be  the  seat  of  a  neuritis  like  that 
which  underlies  sciatica.  The  anterior  crural,  or  a  branch,  suffers 
most  frequently,  with  resulting  symptoms  in  the  front  of  the  thigh, 
or  in  the  outer  part.  These  are  chiefly  sensory  ;  motor  symptoms  are 
seldom  conspicuous  in  cases  so  limited  in  area. 

The  outer  part  of  the  thigh,  in  the  upper  two  thirds,  is  a  specially 
frequent  seat  of  pain  and  occasionally  of  diminished  sensibility. 
Anaesthesia  in  this  region  sometimes  develops  in  a  very  chronic  manner, 
without  pain,  as  an  isolated  but  curiously  enduring  symptom,  usually 
stationary  when  discovered,  and  apparently  the  result  of  a  limited 
neuritis  of  chronic  form.  It  seems  to  have  no  sinister  significance, 
but  has  little  tendency  to  yield  to  treatment. 


NEUEO-MYOSITIS. 


The  term  "  neuro-myositis "  is  perhaps  the  most  convenient  by 
which  to  designate  an  affection,  usually  confined  to  a  single  Hmb, 
generally  the  arm,  in  which  symptoms  indicating  an  interstitial 
rheumatic  inflammation  of  the  lauscles  are  the  leading  feature.  They 
become  tender,  but  the  especial  character  is  the  pain  caused  by  contrac- 
tion of  the  fibres,  i.  e.  by  the  stimulus  which,  normally,  generates 
afferent  impulses.  The  adjacent  fibrous  tissue  may  also  suffer,  and 
especially  that  about  the  joints,  while  the  condition  of  the  nerve  trunks 
may  be  such  as  to  give  rise  to  symptoms  like  those  of  simple  brachial 
neuritis ;  but  in  such  cases  the  affection  of  the  nerves  is  subordinate 
to  the  peculiar  state  of  the  muscles.  The  affection  has  thus  fairly 
well-defined  features  related  to  the  tissue  in  which  the  afferent 
impulses  originate,  and  these  are  common  to  all  cases,  however 
widely  the  rheumatic  affection  may  spread,  and  however  severe  the 


NEURO-MYOSITIS.  185 

additional  symptoms  whicli  partake  of  the  characteristics  of  other 
allied  diseases.  Hence  the  suitability  of  the  name  "  neuro-myositis," 
in  spite  of  some  limitation  of  meaning  which  this  use  implies.* 

The  affection  occurs  chiefly  in  patients  in  the  second  half  of  life, 
and  under  the  same  general  conditions  as  brachial  neuritis.  It  is, 
however,  met  with  mere  frequently  in  young  persons,  sometimes  even 
in  children,  especially  as  a  troublesome  sequel  to  rheumatic  fever, 
limited  to  the  structures  and  muscles  of  the  hand.  More  often  it 
commences  in  the  neighbourhood  of  the  shoulder,  in  the  deltoid  and 
upper  arm  muscles,  and  passes  down  the  arm  to  become  more  severe 
in  the  hand  than  at  the  elbow.  Slight  changes  usually  occur  in  the 
articulation  of  the  shoulder,  but  more  troublesome  arthritic  adhesions 
form  in  the  finger-joints  and  at  the  wiist,  as  is  common  when  mus- 
cular pain  interferes  with  movements  in  rheumatic  persons.  This 
lasts  so  long  in  the  intrinsic  muscles  of  the  hand,  and  tissues  about 
the  tendons,  that  the  fixation  of  the  joints  becomes  permanent  before 
there  is  time  for  the  restoration  of  the  muscles,  and  some  oedema  in 
the  acute  stage  is  followed  by  the  characteristic  changes  in  the 
nutrition  of  the  skin,  peehng  of  the  epidermis  and  wasting  of  the 
subcutaneous  structures. 

But  the  affection  of  the  muscles  is  the  characteristic  feature.  The 
symptoms  clearly  indicate  a  rheumatic  interstitial  inflammation, 
affecting  the  tissue  in  which  the  afferent  nerves  begin,  and  the 
impulses  they  convey  originate,  perhaps,  in  the  mysterious  "muscle- 
spindles."  In  this  tissue  the  impulses,  unfelt  in  health,  are  generated 
by  tension  and  compression,  and  become  painful  in  some  conditions, 
as  after  cramp.  Then,  if  the  muscle  is  stretched  in  extension,  or 
the  interstitial  structures  are  compressed  by  muscular  contraction, 
considerable  pain  is  readily  produced.  Simple  muscular  rheumatism, 
transient,  such  as  the  common  form  of  lumbago,  seems  an  affection 
of  this  interstitial  tissue  too  slight  to  persist  as  permanent  inflam- 
mation. The  chief  feature  of  the  form  now  under  consideration 
is  the  production  of  pain  by  those  forms  of  stimulation  to  which 
the  nerve-endings  normally  respond  (see  p.  58),  compression  by 
voluntary  contraction,  and  the  passive  tension  in  the  antagonist. 
Pain  is  caused  also  in  the  structures  about  the  joints,  especially  in 
the  capsules,  when  the  limit  of  movement  is  reached  which  the 
state  of  the  various  structures  permits.  Even  passive  movement 
induces  a  sudden  contraction  of  the  muscles,  i  artly  reflex,  partly 
voluntary,  which  causes  pain.     This  pain  spreads  widely  in  the  mus- 

*  It  has  indeed  been  proposed  by  Senator  to  use  it  to  designate  another  disease, 
the  rare  acute  symmetiical  myositis  (described  further  on),  whicli  is  like  multiple 
neuritis  in  toxic  causation,  and  distal  bilateral  distribution  ('  Deut.  med.  Wochen- 
schr.,'  1893).  The  term  has  not,  however,  come  into  use  for  this  affection,  and 
seems  not  needed  for  it.  "  Acute  myositis  "  or  "polymyositis,"  the  names  employed 
in  Germany,  are  sufficiently  adequate  to  leave  to  its  jiPL'seut  application  the  term 
"  iRuro-myositis." 


136  NEURO-MYOSITIS. 

cles,  as  tliose  adjacent  contract  to  stop  tlie  movement  to  which  the 
pain  seems  due,  and  it  is  produced  with  readiness  in  the  morbid 
state.  Hence  arises  the  characteristic  feature.  Muscular  contraction, 
the  great  cause  of  pain,  results  from  any  movement,  active  or  passive, 
and  the  patient  acquires  the  conviction  that  no  passive  movement  of 
the  limb  is  possible  without  the  production  of  pain  in  the  muscles. 
Such  pain  does  actually  occur  whenever  an  attempt  is  made  to  move 
the  joints,  as  the  shoulder  and  elbow,  even  in  slight  degree,  or  the 
wrist-joint,  if  with  any  suddenness.  It  is  first  and  most  felt  not  in 
the  neighbourhood  of  the  joints,  but  in  the  muscles.  Afterwards 
some  may  be  felt  in  the  structures  about  the  joint.  On  close  and 
careful  observation  it  will  be  found  that  the  pain  is  related  much  more 
to  the  expectation  of  passive  movement  than  to  that  which  takes  place. 
Before  any  movement  is  produced,  the  muscles  contract  in  order  to 
control  and  restrict  the  source  of  expected  suffering.  The  pain 
coincides  with  this  contraction,  which  is  unconsciously  vohtional  and 
reflex,  rather  than  really  voluntary.  But  with  the  help  of  some 
patience  and  perseverance  it  is  possible  to  get  the  limb  quite  passive, 
resting  without  effort  in  the  observer's  hands,  and  to  make  gentle 
slow  passive  movement  of  the  joints  without  the  occurrence  of  this 
inhibitory  contraction  of  the  muscles.  The  patient  is  often  astonished 
at  the  painless  movement  of  joints  when  he  thought  great  suffering 
would  result  from  the  least  displacement  of  the  parts.  But  if  then 
a  more  sudden  movement  is  made,  and  the  alarm  and  perhaps  slight 
pain  cause  sudden  spasmodic  muscular  contraction,  the  pain  felt 
in  the  muscles  is  great.  There  is  usually  some  tenderness  on  sudden 
compression  of  the  muscular  substance,  and  this  may  be  considerable 
and  produced  with  some  readiness,  but  often  the  sensibility  to 
pressure  is  inconspicuous.  We  know,  from  the  state  left  by  common 
cramp,  how  easily  pain  is  produced  in  the  muscle  by  contraction; 
when  this  is  sudden  and  considerable  the  pain  may  be  shared  by 
the  nerves  ;  as  already  stated,  those  of  the  brachial  plexus  above  the 
clavicle  and  in  the  axilla,  present  the  same  tenderness  as  is  met  with 
in  simple  brachial  neuritis,  although  in  slight  and  subordinate  degree, 
manifestly  from  the  inflammation  of  their  sheaths.  Other  structures 
suffer,  as  already  stated.  The  res\ilt  is  a  considerable  amount  of  fixa- 
tion of  the  limb  from  the  pain  which  is  produced  by  movement ;  this 
has  more  than  one  source,  and  it  may  be  difiicult  at  first  to  discern 
that  from  which  the  chief  suffering  proceeds.  The  arthritic  changes 
within  the  joints  produce  adhesions  which,  with  the  alteration  in  the 
capsule,  fix  the  joint  in  an  abnormal  posture,  generally  that  which  falls 
short  of  complete  extension.  The  contraction  of  the  muscles  to  inhibit 
movement  tends  to  be  more  and  more  persistent  and  continuous,  and 
this  state  of  rigidity  is  prone  to  cause  some  shortening  of  their 
stractures ;  nutritional  change  fixing  the  functional  states  assists  in 
producing  the  slight  deformity  and  fixed  posture  of  the  joints  that 
are  frequently  observed. 


^'EUKO-MYOSITIS.  137 

The  malady  is  commonly  of  very  prolonged  duration,  developing 
insidiously  even  after  recovery  from  an  initial,  more  acute  attack  of 
local  rheumatism,  especially  wlien  slight  persistent  pain  on  move- 
ment is  maintained  by  the  neglect  of  needed  rest.  The  affection  is 
usually  unsymmetrical,  affecting  one  side  only,  after  the  fashion  of  these 
rheumatic  affections  of  the  fibrous  tissues,  of  sciatica,  or  of  brachial 
neuritis.  Nevertlieless,  in  moderate  typical  form,  tlie  manner  in 
which  it  presents  itself  as  an  affection  of  the  interstitial  tissue  of  the 
muscles  and  their  nerves  justifies  the  view  which  is  expressed  in  the 
designation  given  to  it ;  and  the  peculiar  implication  of  the  afferent 
muscle-nerves,  in  consequence  of  its  interstitial  localisation  in  the 
muscles,  justifies  its  inclusion  among  the  maladies  here  described. 

This  is  further  warranted  by  the  condition  which  commonly  results. 
The  duration  of  the  affection  and  the  changes  which  ensue  in  the  sub- 
stance of  the  muscles  and  the  tissue  about  the  joints,  and  the  fixation 
of  these  during  the  time  that  elapses  before  the  cessation  of  pain 
permits  freedom  of  movement,  by  that  time  mechanically  hindered, 
bring  the  cases  into  the  class  of  residual  consequences  of  nerve  lesions. 

The  resulting  interference  with  use  of  the  part  is  greater  than  is 
proportionate  to  the  amount  of  rheumatic  iaflammation,  being  espe- 
cially great  when  much  sensitiveness  has  proceeded  from  the  long- 
continued  pain,  and  fear  of  its  induction  outlasts  the  danger  and 
exceeds  it  in  degree.  Some  chronic  neuralgic  pain  is  apt  to  remain, 
due  in  part  to  the  predisposition  to  neuralgia  which  most  patients  of 
this  class  possess. 

Another  cuiious  sequel  hinders  the  return  of  mobility  in  these  cases. 
Passive  movement  is  difficult  out  of  proportion  to  any  pain  produced, 
although  this  results  if  the  attempt  is  persevered  in  and  its  strength 
increased.  The  common  effect  is  a  peculiar  rigidity  of  the  limb,  such 
as  at  first  suggests  firm  adhesions  at  the  joints — an  impression  that 
may  be  easily  retained  if  the  examination  is  hasty.  Its  nature  is 
rendered  clear  by  sufficient  patience  and  close  observation;  passive 
movements  must  be  attempted  with  the  utmost  gentleness  and  slow- 
ness, and  the  attention  of  the  patient  turned  to  some  other  part  during 
their  course,  while  the  observation  of  the  examiner  remains  fixed. 

The  same  care  is  necessary  in  local  therapeutic  measures  if  these 
are  to  be  successful.  Gentle  upward  rubbing  to  the  muscles  should  be 
afterwards  combined  with  slow  passive  movement,  at  first  scarcely 
appreciable  in  degree,  and  continued  with  the  endeavour  to  keep 
behind  the  production  of  the  muscular  spasm.  This  will  often 
permit  good  to  be  effected  by  local  applications  of  heat,  dry  or 
moist,  liniments,  &c.  The  internal  treatment  needed  is  such  as  is 
suitable  for  the  gouty  and  rheumatic  constitutional  state,  with  tonics 
whore  these  are  indicated,  and  is  of  the  same  general  character  as 
that  recommended  for  brachial  neuritis. 

Gout  and  Rheumatism  in  the  Causation  of  Neuritis. — It  may  seem  to  many 
readers  that  the  range  of  influence  of  these  constitutional  states  has  been  pushed  too 


138  MULTIPLE    NEURITIS. 

far  in  the  preceding  account  of  the  affections,  which  are  primarily  of  fibrous  tissue, 
and  only  of  the  nervous  system  by  reason  of  proximity.  The  difficulty  may  be 
increased  by  the  number  of  patients  who  present  no  adequate  personnl  or  hereditary 
evidence  of  such  causal  disease  or  tendency.  Bat  it  should  be  noted  that  the 
influence  of  gout,  including  ancestral  gout,  is  a  subject  on  which  the  young  practi- 
tioner starts  with  a  high  degree  of  scepticism  regarding  the  teaching  of  his  seniors. 
But  year  by  year  liis  doubts  b-rome  fewer,  as  they  are  rubUed  away,  or  removed 
more  sharply,  by  contact  with    act. 

The  negative  influence  of  the  cases  in  which  the  cause  cannot  be  traced,  or  only  in 
plight  degree,  should  be  carefully  weighed  before  the  result  is  placed  in  the  scale. 


MULTIPLE   NEURITIS. 


The  term  "  multiple  neuritis,"  or  "  polyneuritis,"  is  applied  to  the 
condition  in  which  many  nerves  are  inflamed  simultaneously  or  in 
rapid  succession.  This  multiplicity  is  combined  with  bilateral  sym- 
metry, alike  in  its  seat  and  in  the  special  character  of  the  symptoms. 
These  constitute  its  most  obtrusire  features.  If  only  a  few  nerves 
are  involved,  the  symmetry  becomes  a  more  salient  feature  than  the 
multipHcity.  The  symmetry  is  not  only  characteristic,  it  is  of  profound 
significance,  because  commonly  combined  with  a  third  feature,  the  fact 
that  the  affection  is  most  intense  at  the  extremities  of  the  nerves,  and 
lessens  progressively  towards  the  centre.  This  "  peripheral "  symmetry 
can  only  be  due  to  a  cause  circulating  in  the  blood  or  inherent  in  the 
vitality  of  the  nerves.  The  former  is  the  common  mechanism,  but 
some  influence  of  the  latter  is  indicated  by  this  seat, — farthest  from 
the  central  source  of  nutritional  energy,  where  the  resistance  to  morbid 
influences  is  least. 

On  account  of  the  seat  the  disease  has  also  been  termed  "peripheral 
neuritis,"  a  name  that  is  accurate  but  somewhat  confusing,  because 
it  has  been  used  to  distinguish  all  affections  of  nerve-trunks  from 
central  diseases  causing  similar  symptoms,  especially  in  the  case  of 
the  cranial  nerves.     It  should  not  be  employed  in  this  sense. 

The  name  "  polyneuritis  "  has  also  come  into  extensive  use,  and 
perhaps  will  more  readily  secure  association  with  another  important 
characteristic,  though  practically  identical  with  "  multiple  neuritis." 
The  microscope  shows  that  the  changes  are  almost,  confined  to  the 
proper  tissue  of  the  nerve-fibre,  and  do  not  implicate  the  sheath  or 
interstitial  tissue,  as  a  rule,  even  when  acute.  This,  indeed,  we  might 
expect  from  the  special  limitation  in  the  function  deranged.  But  it 
affords  a  contrast  to  the  forms  of  neuritis  previously  described. 
These  may  be  multiple,  and  even,  very  rarely,  symmetrical,  as  when  both 
sciatic  nerves  are  inflamed.  But  the  symmetry  is  rough,  and  it  does 
not  extend  to  precision  in  the  function  disturbed,  and  soon  ceases  to 
be  distinct.     It  is  this  affecticii  of  the  true  nerve  tissue,  and  its  results, 


PATHOLOGY.  139 

which  marks  the  symmetry  of  the  form  under  consideration,  and  is 
absent  in  the  occasional  multiplicity  of  the  inflammation  met  with 
usually  in  single  nerves.  This  character,  of  absolute  ultimate  import- 
ance, is  more  likely  to  become  attached  to  "  polyneuritis."  A  name 
is  needed  which  embodies  also  the  idea  of  symmetry. 

The  discovery  that  certain  combinations  of  symptoms,  formerly 
thought  to  depend  on  disease  of  the  spinal  cord,  are  really  due  to 
disease  of  the  peripheral  nerves,  is  one  of  the  most  important  steps 
in  the  recent  advance  of  pathology.  It  has  profoundly  modified  many 
of  our  conceptions,  and  has  opened  a  new  field  in  pathology,  the  culti- 
vation of  which  has  but  begun. 

The  occurrence  of  multiple  neuritis  was  first  demonstrated  by 
Dumenil,  of  Eouen  (1864),  although  the  leprous  form  (which  differs 
in  important  pomts)  had  been  previously  described  by  Yirchow. 
Todd  also  deserves  credit  for  a  correct  and  clear  perception  of  an 
affection  of  the  nerves  as  the  cause  of  lead  palsy  in  1854.*  Graves, 
indeed,  long  ago  suspected  that  many  cases  of  paralysis  were  due  to 
disease  of  the  nerves,  but  he  based  his  opinion  on  the  normal  aspect 
of  the  spinal  cord ;  and  in  most  of  the  cases  he  describes  it  is  probable 
that  modern  methods  of  examination  would  have  revealed  disease. 
Symptoms  so  peculiar  had  been  noted  from  time  to  time ;  first, 
perhaps  (1822),  by  Dr  J.  Jackson,  of  Boston,  U.S.A.  They  were 
fidly  described  by  Duchenne  in  1858.  Even  Dumenil's  observations 
attracted  little  notice,  and  it  was  not  until  fresh  facts  were  brought 
forward  by  Joffroy  (1879),  Leyden  (1880),  and  Grainger  Stewart 
(1881),  that  attention  was  generally  directed  to  the  subject.  Since 
that  time  a  very  large  number  of  observations  have  been  published, 
and  the  wide  relations  of  the  morbid  process  are  now  perceived. 

Among  these  one  stands  out  conspicuously.  The  forms  of  multiple 
neuritis  which  present  local  and  functional  symmetry  and  are  of 
parenchymatous  nature  must  be  ascribed  to  a  blood- state  having 
equal  access  to  all  parts,  but  a  special  action  on  those  that  suffer, 
or  do  so  most  readily,  owing  to  a  peculiar  and  constant  disposition 
inherent  in  those  structures  thus  to  be  affected.  The  cause  of  this 
affection  is  the  presence  in  the  blood  of  some  virus,  and  the  sahent 
fact  is  that  the  toxic  agent  is  usually  a  poison  of  chemical  nature,  a 
metal,  or  an  organic  chemical  material  often  resulting  from  the 
growth  of  organisms  in  or  out  of  the  body,  sometimes  apparently  from 
disordered  processes  of  digestion  or  excretion,  or  deranged  metabolism 
within  the  body,  varied  in  seat  and  nature.  To  such  a  poison  some 
nerve-fibres  are  specially  susceptible,  just  as  they  are  to  curara,  or  to 

*  The  first  ascription  of  a  form  of  paralysis  to  this  lesion  was  by  Dr.  Todd,  in  the 
case  of  lead  palsy.  "  I  believe  that  the  muscles  and  nerves  are  early  affected,  and 
that  at  a  later  period  the  nerve-centres  become  implicated.  The  nervous  system  ia 
thus  first  affected  at  its  ppriphery,  in  the  nerves,  and,  the  poisoning  influence  con- 
tinuing, the  contamination  gradually  advances  towards  the  centre  "  (*  Clin.  Lect./ 
1854,  p.  9). 


l40  MULTIPLE    NEURITIS. 

strycliuia,  or  to  atropia.  It  is  a  manifestation  equally  of  a  peculiarity 
in  the  acting  virus  and  in  the  structures  acted  on.  The  virus  is  clearly 
one,  in  most  cases,  of  chemical  composition ;  and  it  may  be  so  also  with 
the  chanL,^es  in  structiires  acted  on — under  the  influence  of  life  incon- 
ceivably minute  but  constant,  and  determining  the  changes  which 
attend  functional  action,  and  modifying  this  when  altered. 

Isolated  neuritis  may  be  caused  in  part  by  a  constitutional  or  blood 
state,  but  is  not  due  to  this  alone.  An  exciting  cause  is  in  opera- 
tion, which  acts  locally  and  determines  the  position  of  the  affection. 
Hence,  as  we  have  seen,  such  neuritis  is  rarely  multiple,  and  still 
more  rarely  even  roughly  symmetrical,  while  these  features  are  ex- 
plained by  the  irregular  affection  of  the  connective  tissue  instead  of 
an  impaired  nutrition  of  the  proper  neural  structures. 

This  distribution  holds  good  to  a  remarkable  extent  and  degree. 
When  the  symmetrical  polyneuritis  is  severe,  even  to  death,  and  the 
nerve- structure  has  practically  disappeared,  by  destructive  change,  from 
the  distal  parts  of  certain  nerves,  the  interstitial  tissue  and  sheath 
show  only  a  trifling  increase  in  their  nuclei.  "We  find  morbid  appear- 
ances in  them  chiefly  in  extremely  acute  cases,  when  the  vessels  are 
dilated  in  the  sheath  or  interstices ;  the  changes  due  to  oedema  are 
seen ;  white  corpuscles  are  aggregated  outside  the  minute  vessels, 
and  even  extravasations  of  blood  occur  in  addition  to  rapid  destruc- 
tion of  the  fibres  proper.  So,  on  the  other  hand,  a  very  intense 
interstitial  neuritis  may  involve  the  nerve- fibres  proper. 

There  are  also  cases  in  which  the  causes  of  the  two  forms  are  com- 
bined. Alcoholism,  for  instance,  the  most  common  toxic  cause,  may 
be  due  to  influences  that  have  also  produced  gout,  and  then  isolated 
gouty  perineuritis  may  accompany  polyneuritis  of  parenchymatous 
nature  and  symmetrical  distribution.  In  one  instance  of  this  a  peri- 
neuritis of  one  external  popliteal  nerve,  causing  palsy  of  the  flexors 
of  one  ankle-joint,  limited  to  that  side,  was  followed,  after  some 
months  (its  cause  continuing),  by  a  gradual  loss  of  power  in  these 
muscles  on  both  sides,  with  the  reaction  of  degeneration  in  the 
muscles,  loss  of  the  knee-jerk,  pains,  and  sensory  changes.  Exposure 
to  cold,  again,  may  cause  a  "  rheumatic "  perineuritis,  either  of  a 
single  nerve  or  of  a  few  nerves  irregularly  distributed,  and  it  may 
also  produce  a  blood- state  allied  to  that  of  acute  rheumatism,  and  a 
symmetrical  parenchymatous  polyneuritis  sometimes  most  severe. 
The  effective  poison  may  be  due  to  organisms  in  the  blood,  especially 
those  of  specific  nature  that  cause  a  definite  disease,  as  diphtheria  or 
smallpox.  They  may  be  of  less  specific  character,  as  those  which 
enter  by  contaminated  wounds,  impure  puerperal  processes,  and  the 
like,  the  effects  of  which  may  be  severe  out  of  all  proportion  to  their 
apparent  cause,  from  a  special  susceptibility  to  certain  agents.  We 
have  reason  to  ascribe  the  neuritis  to  chemical  agents  produced  by  the 
organisms,  but  they  seem  themselves  capable,  in  many  cases,  of  setting 
up  neuritis  also,  interstitial  in  seat  and  moi'e  irregular  in  'dicLribution, 


ETIOLOGY.  141 

besides  giving  rise  to  tlie  product,  perhaps  a  chemical  snbstance,  com- 
parable to  alcohol,  capable  of  causing  a  parenchymatous  neuritis. 
The  two  foiins  may  then  be  conjoined  in  a  puzzling  manner,  varying 
in  relative  amount  in  different  cases.  It  is  probably  thus  that  the 
forms  of  diffuse  or  total  neuritis  are  produced,  occasionally  met  with 
in  diphtheritic  paralysis.     We  have  here  a  field  still  to  be  investigated. 

Not  less  important  and  mysterious  are  the  distinct  indications  that 
symmetrical  neuritis  results  from  toxines  developed  within  the  body 
from  the  effects  of  other  chronic  diseases.  Diabetes  is  one  of  these, 
but  the  poisonous  agent  is  not  the  sugar  in  the  blood,  for  the  palsy 
occurs  when  there  is  but  a  very  small  amount  in  the  urine.  One  de- 
ranged chemical  process  involves  others,  which  ^ary  in  different  persons, 
and  minute  chemical  variations  may  determine  profound  differences 
in  effect,  ranging  from  the  innocuous  to  the  toxic.  In  advanced 
organic  kidney  disease  also  signs  of  polyneuritis  may  appear — pains  in 
the  legs,  loss  of  the  knee-jerk,  paralysis  of  the  muscles,  tingling  of  the 
skin,  anaesthesia.  Poisons  capable  of  producing  the  condition,  but  of 
unknown  nature,  seem  sometimes  to  be  taken  in  from  the  outside, 
although  we  know  not  how,  for  cases  of  characteristic  features  occur 
when  the  most  exhaustive  search  fails  to  discover  a  known  cause. 

Lastly,  symptoms  of  this  state,  moderate  in  degree — weakness  at 
the  ankle,  loss  of  the  knee-jerk,  subjective  sensations,  or  definitely 
defective  sensibility  below  the  knees,— sometimes  come  on  gradually 
in  the  old,  without  other  cause  than  the  influence  of  advancing  years. 
The  symptoms  seem  not  to  be  progressive,  and  become  less  noticed 
when  they  are  slight,  although  they  are  sometimes  maintained  by 
attention  in  a  troublesome  degree.  They  are  apparently  due  to  a 
degenerative  tendency  due  to  age,  and  are  signs  of  a  senile  form.  We 
must  assume  that  the  nerves  which  suffer  most  in  this  disease  possess 
less  power  than  others  of  resisting  morbid  agencies,  and  that  their 
capacity  for  vital  endurance  varies  in  different  individuals,  to  under- 
stand the  occurrence  of  the  malady  in  those  who  suffer,  and  the 
greater  readiness  with  which  it  is  caused  in  some  by  a  small  quantity 
of  the  poison.  Indeed,  we  are  confronted  at  the  outset  by  a  fact 
which  involves  such  a  difference  inherent  in  the  two  sexes.  The  most 
common  cause,  alcohol,  gives  rise  to  polyneuritis  far  more  frequently 
in  women  than  in  men.  Half  a  dozen  cases  will  be  met  with  in  females 
before  one  is  seen  in  a  man.  An  excessive  degree  of  such  a  disposi- 
tion to  degenerate  would  necessarily  involve  an  earlier  manifestation 
of  the  imperfect  nutrition  which  is  the  normal  termination  of  life. 

The  discernment  of  this  symmetrical  derangement  of  function,  of 
its  dependence  on  the  nerves,  and  the  action  on  them  of  morbid 
agents  conveyed  by  the  blood,  has  enlarged  our  pathological  concep- 
tion by  revealing  the  natui-e  of  cases  previously  misconceived,  because 
absolutely  not  common,  and  differing  too  widely  in  those  features  that 
would  be  put  together  for  their  nature  to  be  readily  recognised. 
Indeed,  their  recognition  is  probably  one  of  the  results  of  the  per- 


142  MULTIPLE    NEURITIS. 

ception  of  how  much  is  meant  by  the  disappearance  of  the  knee-jerk, 
and  the  need  to  observe  minutely  and  compare  carefully  all  cases  in 
which  it  disappears.  This  was  at  least  one  cause  for  the  appreciation 
of  the  significance  of  the  isolated  palsy  of  the  flexors  of  the  ankle 
coinciding  Avith  such  loss  as  it  was  found  to  do,  and  the  more  careful 
study  of  such  eases  to  which  the  observation  led. 

But  the  discovery  of  the  relation  of  the  affection  of  the  nerves  to 
alcohol,  which  swiftly  followed  the  perception  of  its  peculiar  features 
and  the  character  of  their  cause,  has  had  effects  of  even  greater  import- 
ance, and  an  influence  on  our  discernment  still  far  from  exhausted. 
Alcohol  is  a  purely  chemical  agent.  Yarious  metallic  poisons,  acting 
in  minute  amount  and  slowly,  were  also  found  to  have  similar  effects, 
while  in  other  cases  the  toxines  seemed  to  be  also  organic  chemical 
substances.  This  was  also  supplemented  by  the  discovery  that  de- 
rangement of  the  nervous  system  such  as  occurs  in  tetanus  was 
due  to  substances  of  similar  nature,  resembling  strychnia  in  effect  (a 
vegetable  alkaloid),  and  yet  generated  by  organisms  in  the  system; 
that  the  cause  of  diphtheritic  palsy  was  also  a  chemical  substance, 
a  modified  albuminose;  and  that  a  similar  agency  is  the  cause  of 
the  polyneuritis  which  may  follow  other  acute  specific  diseases.  A 
wide,  unstudied  field  of  chemical  pathology  has  thus  been  opened,  on 
which  multi]3le  neuritis  will  find  a  place,  and  clearly  included  therein 
are  the  forms  which  result  from  disordered  processes  within  the  body 
in  the  subjects  of  gout,  exposure  to  cold,  and  the  like.  Chemical 
change  in  the  nerve- tissue  attends  all  its  functions,  and  by  their 
activity  the  renewal  of  elements  is  increased.  Its  character  depends 
on  the  composition  of  the  j)lasma  bathing  them,  and  containing  the 
molecules  to  replace  those  broken  up  in  function.  A  slight  difference 
in  the  constitution  of  the  replacing  molecules  seems  merely  to  derange 
the  process  at  first,  and  a  trifling  alteration  in  function  is  the  only 
result,  such  as  subjective  tingling  when  the  sensory  nerves  suffer 
chiefly,  or  lessened  perception  of  the  most  gentle  stimuli.  The  per- 
sistence of  the  process  leads  to  graver  change  in  molecalar  composi- 
tion and  more  serious  effects,  and  at  last  the  change  amounts  to  an 
alteration  which  may  abolish  function  and  be  visible  under  the 
microscope.  Such,  for  example,  is  the  effect  of  arsenic ;  typical 
polyneuritis  results  from  the  constant  presence  of  a  definite  quantity 
in  the  blood.  Arsenic  has  chemical  correspondences  with  phosphorus, 
as  well  as  differences,  which  enable  us  to  understand  its  assimilation 
by  the  nerves,  and  also  the  gradual  changes  that  result,  molecular  at 
first,  but  at  last  structural.  The  affinity  for  certain  parts  of  the 
nervous  system  presented  by  different  substances  enable  us  also  to 
understand  that  acute  arsenical  poisoning  may  be  followed  by  a 
similar  chronic  neuritis  of  bilateral  and  peripheral  distribution,  and 
both  motor  and  sensory  symptoms,  while  very  chronic  poisoning 
causes  more  various,  effects,  apparently  determined  by  dose  and  the 
combinations  which  the  state  of  the  system  causes  it  to  form,  and  in 


ETIOLOGY.  143 

which  it  is  presented  to  the  nerves.  The  specialisation  of  the  sensory 
symptoms  is  remarkable  in  its  variations  from  all  causes ;  one  form  of 
sensation  may  alone  be  disturbed.  Always,  however,  the  form  is  the 
same,  and  the  seat  is  the  same  on  each  side  ;  and  this  feature,  whether 
the  disturbance  is  trifling  or  grave,  should  arouse  a  suspicion  that  we 
have  to  do  with  the  action  on  the  nerves  of  a  toxic  agent  in  the  blood. 
The  importance  of  this  indication  cannot  be  insisted  on  too  often  or  too 
strongly.  It  will  often  save  from  serious  error,  from  loss  of  precious 
time,  and  the  patient  from  much  suifering  and  prolonged  disability. 

In  both  acute  and  chronic  forms  the  influence  of  the  toxic  agent  may 
be  exerted  on  the  central  organs  as  well  as  on  the  peripheral  nerves, 
not  only  so  as  to  give  rise  to  more  or  less  diffuse  inflammation,  but 
limited  degeneration  of  tracts  of  fibres,  as  of  the  posterior  columns  or 
the  pyramidal  tracts.  The  frequent  relation  of  the  former  to  a  toxine 
is  well  known,  and  degeneration  of  the  lateral  tracts  is  known  some- 
times to  begin  at  the  extremities  of  the  fibres  in  a  way  analogous  to 
that  of  the  peripheral  nerves.  It  is  not,  therefore,  surprising  to  find 
central  symptoms  combined  with  those  of  neuritis  in  various  forms, 
and  the  resulting  effects  may  present  characters  of  a  puzzhng  aspect. 
Such  wider  distribution  must  be  ascribed  to  the  predisposition  of  the 
subject  and  the  character  of  the  active  agent.  It  is  indeed  surprising 
that  the  effects  of  this  one,  so  frequently  limited  as  they  seem  to  be, 
and  the  common  restriction  of  their  incidence,  are  significant  of  a 
definite  uniform  difference  in  the  chemical  relations  of  the  nerve- 
structures,  no  doubt  with  an  absolute  relation  to  function  imder 
normal  conditions  as  well  as  to  disease.  Indeed,  the  physiological 
importance  of  the  facts  of  toxic  neuritis  are  most  distinct.  They  sug- 
gest that  chemical  processes  have  a  larger  share  in  determining  those 
of  vital  function  than  has  been  thought,  and  that  their  range  may  be 
found  more  extensive  and  important  than  was  previously  suspected. 

An  important  question  has  been  raised  by  the  discovery  that  the 
central  structures  often  sirffer  in  cases  of  peripheral  polyneuritis,  and 
that  some  toxic  agents  seem  to  act  also  on  the  nerve-cell,  the  central 
element  of  the  neuron,  as  well  as  on  the  peripheral  structures.  But 
the  facts  are  still  too  scanty  to  be  of  use,  and  will  be  referred  to 
again  in  the  section  on  pathology. 

It  is  to  the  pathology  of  the  disease  that  we  must  look  for  its 
effective  subdivision,  but  our  knowledge  of  the  subject  is  still  imper- 
fect. The  causes  of  polyneuritis,  numerous  and  various  as  they  are, 
stUl  furnish  the  best  groimd  for  a  practical  classification  of  the  forms 
of  the  disease  such  as  will  assist  us  in  recognising  them  and  in 
treating  them. 

Symmetrical  regularity  means  a  blood- state  acting  alone ;  irregular 
multiplicity  generally  means  a  constitutional  state  combined  with  local 
determining  causes.  When  very  few  nerves  are  affected  irregularly, 
the  general  influence  sinks  to  the  level  of  a  predisposition,  and  this  is 
not  always  to  be  recognised  when  a  single  nerve  is  affected. 


144  MULTIPLE  NEURITIS. 

The  acuteness  or  slowness  of  the  onset  has  also  been  made  a  ground 
of  distinction,  but  the  symptoms  vary  much  in  aspect  and  grouping 
according  to  their  intensity.  The  more  chronic  the  process  the  more 
perfect  is  the  limitation  of  the  process  and  of  its  effects  on  function. 
In  the  most  chronic  form  the  process  in  the  nerve-elements  resembles 
a  pure  degeneration,  and  hence  has  been  termed  "  degenerative 
neuritis."  From  most  causes  we  meet  with  cases  both  acute  and 
chronic,  according  to  the  intensity  of  the  toxic  agent. 

No  arrangement  can  be  free  from  anomalies  until  we  know  more  of 
the  causes  and  of  the  mechanism  by  which  the  varieties  are  produced ; 
but  provisionally,  therefore,  that  we  may  survey  their  relationship,  the 
cases  may  be  placed  in  the  following  groups. 

It  will  be  instructive  to  survey  them  first  generally ;  some  wiD. 
need  afterwards  more  special  consideration. 

I.  Toxic  :  due  to  the  presence  in  the  blood  of  a  known  poison,  a  substance 
commonly  so  described  ;  of  such  the  simplest  is — 

(a)  Metallic :  lead,  silver,  &c. 

(6)  Non-metallic,  as  alcohol — the  most  frequent  cause.     Other  or- 
ganic   substances,    formed   outside   the   body,   comparatively 
simple  and  well  known,  act  in  a  similar  manner. 
II    Toxemic:  due  to  some  virus  in  the  blood,  the  precise  nature  of  which  is 
unknown,  but  which  is  apparently  a  chemical  substance  formed  within 
the  body.     It  may  be — 

(a)  Pathogenetic,  the  consequence  of  a  specific  disease,  probably  due 
to  the  growth  within  the  body  of  the  organisms  of  the 
disease,  as  poisons  causing  symptoms  of  another  class  are  due 
to  the  organisms  of  tetanus.  Hence  the  polyneuritis  which 
may  succeed  the  acute  specific  fevers.  A  poison  having  similar 
effects  results  from  the  organisms  of  septicsemia  in  its  varied 
forms,  traumatic  and  other,  causing  most  of  the  cases  of  puer- 
peral neuritis. 

(6)  Metabolic. — A  derangement  of  the  chemical  processes  of  the  body 
may  give  rise  to  a  toxine  capable  of  acting  on  the  nerves.  It 
may  be  the  only  product  of  the  derangement  that  manifests  its 
presence  by  symptoms,  but  other  abnormal  substances  ai^e 
formed  which  can  be  readily  detected,  or  give  rise  to  other 
obtrusive  symptoms.  Thus  peripheral  neuritis  occurs  in 
diabetes,  not  from  the  excess  of  sugar  in  the  blood,  but 
from  some  other  chemical  substance  the  nature  of  which  is  not 
yet  known.  We  cannot  have  a  disorder  of  the  chemical  pro- 
cesses that  causes  an  excess  of  one  substance  without  other 
materials  being  produced  abnormal  in  their  nature  or  in  their 
amount.  There  may  be  known  the  characteristics  of  some 
malady,  of  which  the  neuritis  is  an  apparent  consequence;  or 
they  may  be  inconspicuous,  causing  no  distinct  effects,  and  the- 
neuritis  develops  as  the  only  consequence  of  the  causal  derange- 
ment. These  forms  may  be  termed  secondary  and  primary 
metabolic  neuritis,  according  as  other  abnormal  products  mani- 
fest  their  presence  or  are  latent,  but  there  is  evidently  na 
essential  difference  between  the  two  classes. 


etioloPtT.  145 

(e)  Rhetimatic. — An  important  variety  of  this  class  is  that  in  which  a 
toxine,  seemingly  of  corresponding  nature,  results  from  an 
external  influence,  such  as  exposure  to  cold.*  Rheumatic  poly- 
nenritis  is  a  special  form,  and  yet  seems  to  be  a  metabolic  form 
acutely  excited  by  a  process  the  occurrence  of  which  we  fan 
eee,  but  cannot  understand.  It  varies  much  in  its  intensity, 
and  in  the  severity  and  character  of  the  attendant  catarrhal  or 
rheumatic  symptoms.  They  may  amount  to  those  of  aotual 
accompanying  disease,  which  may  assume  predominant  inten- 
sity and  bear  the  aspect  of  a  primary  affection  to  which  tlie 
neuritis  seems  to  be  secondary,  and  a  consequence  rather  than 
a  mere  concomitant.  But  its  cause  is  evidently  the  presence 
of  an  agent  in  the  hlood  capable  of  deranging  -acutely  the 
nutrition  of  the  nerves  in  a  manner  more  or  less  grave  and 
endui'ing.  The  morbid  agent  thus  generated  must  be  pro- 
duced by  a  disordered  action  occurring  under  the  influence  of 
the  exposure,  but  in  consequence  of  a  pre-existing  state  of  the 
system,  which  we  can  best  conceive  as  the  presence  in  it 
cf  some  morbid  state,  most  probably  of  the  nature  of  a 
chemical  material,  abnormal  in  character,  although  perhaps 
differing  but  little  from  those  that  are  normally  present.  It 
may  be  more  closely  allied  to  such  products,  although  we  con- 
ceive it  most  readily  as  analogous  to  a  simple  poison. 

(cl)  Septicsemic. — From  such  cases  we  pass  to  others  in  which  the 
cause  seems  to  be  more  special  in  nature,  and  in  which  the 
resulting  disorder  of  .the  peripheral  nerves  is  part  of  the 
general  disturbance  of  septicaemia.  Septicsemic  polyneuritis 
constitutes  an  important  variety,  frequent  and  often  most 
grave.  Its  causes  are  numerous,  but  present  two  important 
variations  in  nature;  the  cause  may  be  an  injury  or  operation, 
or  it  may  be  of  entirely  internal  nature.  An  infective  process 
is  the  source  of  an  organismal  mechanism  in  many  cases  of 
this  class.  To  it  belong  most,  perhaps  all  instances  of  puer- 
peral polyneuritis,  an  important  group,  which  vary  in  nature, 
however,  so  as  to  make  their  precise  classification  very  difficult. 
They  present  the  common  symptoms  in  characteristic  and 
usually  in  acute  form,  but  these  may  begin  before  or  after 
delivery  in  association  with  other  symptoms  of  blood-poisoning 
although  they  predominate  in  the  cases  that  can  properly  be 
placed  in  this  category.  But  variations  in  course  are  some- 
times conspicuous,  and  cases  are  seen  which  present  no  evidence 
of  distinct  causation.  Without  doubt,  however,  the  majority 
are  due  to  some  form  of  septicsemic  process,  and  this  is  true 
when  other  signs  of  it  are  inconspicuous.  Puerperal  poly- 
neuritis is  septicsemic  as  an  almost  invariable  rule.  Rare 
cases  are  met  with,  however,  in  which  the  symptoms  of  neuritis 
develop  insidiously,  and  it  may  be  some  time  before  delivery, 
in  a  manner  that  increases  their  mystery,  while  this  is  further 
augmented  by  the  termination  of  these  cases  in  recovery. 


*  See  Ross  and  Judson  Bur)''s  *  Treatise  on  Peripheral  Neuritis.' 
VOL.   I.  10 


14fi  MULTIPLE    NEURITIS. 

£11.  Organismal  :  of  similar  character,  indeed,  are  the  cases  of  the  disorders 
that  eoTisist  in  an  inflammation  of  the  peripheral  nerves,  although  its 
cause  sfeiiis  to  he  the  product  of  the  growth  of  organisms  outside  or 
inside  the  system.  This  view  is  the  best  we  can  take  of  the  pathology 
of  such  a  disease  as  beri-beri.  Even  in  the  case  of  leprosy,  in  which 
there  is  proof  of  the  presence  of  organisms  as  the  material  agents  of  the 
affection,  we  liave  also  strong  reason  to  regard  the  symptoms  as  largely 
the  result  of  the  influence  on  the  nerve-structures  of  a  chemical  agent 
or  agents  which  are  produced  by  these  in  their  growth.  The  indirect 
evidence  of  the  chemical  nature  of  the  toxine  which  acts  on  the  nerve- 
fibres,  and  of  its  organismal  source,  becomes  very  strong  when  the 
known  facts  are  compared  with  the  proofs  we  have  that  such  an  agent, 
so  produced,  is  that  which  gives  rise  to  the  symptoms  of  tetanus  by 
setting  up  a  process  of  local  inflammation,  and  note  that  in  each  instance 
there  is  a  selective  action  upon  certain  structures. 

IV.  Idiopathic  roLTNECEiTis,  we  may  term  the  variety  in  which  no  ex- 
ternal influence  acts  on  the  nerve-fibres;  their  nutrition  fails  spon- 
taneously. We  have  this  condition  best  marked  in  the  important 
variety  in  which  the  symptoms  of  sensory  neuritis  come  on  in  late  life, 
in  consequence  of  an  imperfection  in  the  endurance  of  the  nutrition  of 
the  nerve-fibres.  This  seems  to  depend  upon  a  general  constitutional 
imperfection  which  manifests  itself  in  this  particular  direction  for  some 
reason,  the  nature  and  precise  cause  of  which  we  cannot  discern.  In 
other  cases  we  may  not  have  the  same  special  eifect.  It  is  manifested 
by  the  sensory  moie  than  by  the  motor  nerves,  and  chiefly  as  irritative 
disturbance  of  fumtion. 

We  do  not  usually  meet  with  this  idiopathic  degeneration  except  as  a 
senile  condition.  It  is  true  that  it  may  occur  as  a  condition  that  is  not 
infrequent  although  we  fail  to  recognise  it,  and  we  must  regard  some 
degree  of  disposition  as  underlying  the  occurrence  of  the  morbid  state  in 
the  majority  of  instances  in  which  it  occurs.  Yet  the  fact  that  some 
adequate  causal  influence  is  to  be  traced  so  frequently  as  facts  show  it  to 
be,  reduces  to  small  limits  any  disposition  to  an  idiopathic  failure  of 
nutrition  such  as  has  been  supposed  to  constitute  the  morbid  state  in 
this  form,  and  makes  it  improbable  that  it  plays  any  considerable  part 
in  the  general  production  of  the  symptom. 

Some  of  tliese  causal  varieties  need  more  detailed  consideration. 
The  simple  toxic  forms,  of  wliicli  that  due  to  alcohol  is  the  most 
characteristic,  present  the  most  typical  manifestation  of  the  disorder, 
and  afford  the  ground  for  its  description. 

We  must  separate  from  these  cases  of  symmetrical  bilateral  neuritis 
those  in  which  there  is  an  irregular  affection  of  many  nerves. 

Syphilitic  neuritis  is  an  affection  of  single  nerves,  but  several  may 
■chance  to  suffer  in  the  same  limb.  It  is  a  result  of  the  direct  action  of 
the  organisms  on  the  adventitial  tissues.  True  acute  multiple  neuritis 
has  been  thought  to  be  directly  syphilitic  in  some  cases.  It  would  be 
due  to  an  early  secondary  toxine  produced  by  the  organisms.  The 
evidence  of  it  is  inconclusive.  A  late  polyneuritis  must  be  regarded  as 
the  lesion  of  tabes,  and  this  is  sometimes  met  with  in  an  acute  form. 


ETIOLOGr.  147 

Buggesting  tliat  a  toxine  may  be  produced  at  an  early  stage  of  the 
disease,  capa.ble  of  acting  on  the  nerves  and  causing  polyneuritis. 
The  subject  is  considered  in  the  section  on  Locomotor  Ataxy,  but  it 
is  important  to  mention  it  here,  because  this  disease  presents  an 
example  of  central  and  peripheral  lesions  due  to  the  same  cause,  and 
because  an  affection  of  the  sensory  nerves  almost  identical  in  dis- 
tribution and  nature,  and  giving  rise  to  similar  effects,  is  sometimes 
the  consequence  of  alcohol,  and  occasionally  also  of  other  agents,  as 
arsenic* 

Tubercular  polyneuritis  is  a  form  regarding  which  we  have  still 
much  to  learn.  It  has  occasionally  developed  in  the  course  of  phthisis, 
and  has  been  found  to  be  of  the  purely  parenchymatous  or  degene- 
rative variety.  Severe  cases,  with  characteristic  motor  and  sensory 
symptoms,  in  which  other  causes,  such  as  alcohol,  can  be  excluded,  are 
rare,  but  their  occurrence  is  well  established,t  and  they  seem  to  justify 
the  conclusion  that  the  tubercle  bacillus  is  capable  of  producing  a 
poison  having  a  specific  action  on  the  nerves — a  mechanism  also  indi- 
cated by  the  fact  that  the  bacilli  themselves  cannot  be  found  in  the 
affected  structures.  It  is  said  that  similar  slighter  neuritis  is  frequently 
to  be  foimd  after  death  in  cases  of  phthisis,  and  probably  produces 
symptoms  that  are  overlooked  in  the  general  prostration  of  the  later 
stages  of  the  disease.  Some  severe  cases  recorded  as  tubercular  are, 
however,  not  free  from  the  suspicion  that  alcohol  was  in  part  or  wholly 
the  cause  of  the  neuritis.  There  is,  moreover,  another  relation  between 
multiple  neuritis  and  phthisis ;  the  latter  often  develops  in  the  course 
of  alcoholic  neuritis,  and  is  a  frequent  cause  of  death. 

Qout  gives  rise  to  interstitial  neuritis  and  perineuritis,  usually 
isolated,  but  sometimes  multiple  and  then  irregular  in  distribution. 
Yet  chronic  alcoholism  occasionally  coincides  with  the  gouty  state,  and 
may  cause  a  combination  of  the  symmetrical  toxic  form  of  typical 
character,  and  an  isolated  neuritis,  such  as  inflammation  of  the  sciatic 
nerve — a  combination  which  may  puzzle  unless  this  origin  of  it  ia 
recognised. 

Most  Acute  Specific  Diseases  have  been  followed  by  neuritis,  some 
more  frequently  than  others,  especially  diphtheria,  variola,  measles, 
typhus  and  typhoid  fevers ;  and  influenza  has  had  this  sequel  with 
notable  frequency  during  the  epidemic  of  the  last  five  years.  The 
interval  that  usually  elapses  between  the  acute  disease  and  the  neuritis 
gives  strong  support  to  the  pathology  of  the  disease  above  described. 
The  occurrence  of  the  neuritis  does  not  seem  to  be  related  to  the 
severity  of  the  primary  disease,  but  rather  to  some  peculiarity  in  the 
special  organisms,  such  as  so  often  determines  peculiar  features  in  an 

*  Durdufi  ('Neur.  Cent.,*  1893,  p.  632)  directs  attention  to  a  fact,  especially 
insisted  upon  by  Leyden,  Gowers,  and  others,  that  gonorrhoea  may  give  rise  to 
morbid  conditions  of  the  nervoas  syatem,  and  mentions  especially  neuritis  and 
myelitis. 

t  See  Oppenheim,  *  Zeitschr.  f .  klin.  Med.,'  1886,  p.  230. 


148  MULTIPLE    NEURITIS. 

epidemic.  This  feature  is  conspicuous  in  all  the  acute  specific  diseases. 
In  one  case,  which  developed  six  weeks  after  varioloid,  with  pains  in 
the  limbs,  atrophy  of  the  arms  and  legs,  &c.,  the  purely  neuritic  nature 
of  the  morbid  process  was  ascertained  six  months  later,  when  the 
patient  died  from  pneumonia.  But  the  mechanism  is  apparently  more 
complete  in  some  maladies.  In  diphtheria  a  poison  is  produced  which 
has  a  more  extensive  action  on  the  nerve-structures.  This  is  described 
in  more  detail  in  the  second  volume.  In  this  disease,  moreover,  the 
acute  stage  is  attended  by  an  acute  inflammation  of  all  the  elements  of 
the  nerve- structures,  and  not  merely  of  the  functional  parts — a  more 
intense  morbid  agent  being  apparently  active.  Malarial  neuritis  seems 
to  occupy  a  position  intermediate  between  the  secondary  and  primary 
forms,  since  it  sometimes  follows  malarial  fever,  and  sometimes  deve- 
lops without  previous  fever  in  those  who  have  been  exposed  to  the 
influence  of  malaria.  We  have  much  still  to  learn  regarding  the 
pathology  of  these  forms. 

Septicsemic  polyneuritis  is  a  rare  variety,  but  one  of  the  most 
formidable  in  many  cases,  the  nerves  to  vital  organs  suffering  with 
relative  frequency  and  severity.  The  most  severe  cases  have  been 
traumatic,  the  result  of  organisms  introduced  from  without  into 
some  wound,  or  of  some  injury  or  local  inflammation  capable  of 
giving  rise  to  blood-poisoning.  In  some  such  cases  the  disease  has 
been  unaccompanied  by  the  usual  symptoms  of  septic  blood-states, 
but  has  followed  its  cause  at  an  interval  that  suggests,  taken  with 
other  analogous  facts,  that  it  is  due  to  some  virus  produced  pro- 
bably by  septic  organisms  of  a  special  character.  For  instance,  a 
man,  thirteen  days  after  a  stab-wound  beneath  the  clavicle,  which 
healed  well,  had  an  attack  of  parotitis  with  facial  palsy.  On  the 
fortieth  day  there  developed  paralysis  of  tongue,  vocal  cords,  and 
limbs,  and  on  the  sixth  day  after  the  onset  of  these  symptoms  he  died 
from  respiratory  palsy.  Extensive  peripheral  neuritis  was  the  only 
nerve  lesion.*  In  a  less  severe  case  recorded  by  Barrsf  the  symptoms 
commenced  three  weeks  after  a  neglected  wound  of  the  hand,  which 
produced  inflammation  of  the  lymphatics,  extending  up  the  arm  to  the 
axilla.  The  symptoms  of  neuritis  began  in  the  injured  limb,  and 
invaded  successively  the  other  extremities,  causing  characteristic  weak- 
ness in  the  lower  limbs,  with  loss  of  the  knee-jerk.  He  slowly 
recovered.  It  is  possible  that  some  cases  of  this  class  result  from 
unsuspected  foci  of  inflammation  within  the  body,  in  which  pus 
becomes  pent  up  and  causes  the  blood- state. 

•  In  cases  with  a  possible  internal  source  of  blood-poisoning,  poly- 
neuritis may  develop  as  an  apparently  primary  disease  if  the  source  of 
the  septicaemia  has  been  undiscovered.  The  nature  of  such  cases  is 
often  mysterious.  They  are  not  separable  from  those  due  to  deranged 
metaboHsm,  or  to  some  toxine  arising  by  a  different  mechanism. 

•  Roth,  '  Corr.-Bl.  f.  Schw.  Acrzte,'  1883,  No.  13. 
t  'Amer.  Jnurn.  Med.  Science,'  Feb.,  1889. 


ETIOLOGY.  149 

Other  primary  cases  occur,  moreover,  in  whidi  tHere  is  no  cause  for 
septicaemia,  and  yet  of  sucli  severity  as  to  suggest  a  poison  coming 
from  a  powerful  external  source.  The  only  explanation  we  can  give  of 
these  is  that  they  are  probably  due  to  a  virus  received  from  without, 
the  precise  nature  of  which  is  unknown,  and  that  there  are  sources 
of  such  toxines  of  which  we  are  still  ignorant.  Some  cases  have  been 
met  with  in  which  the  symptoms  of  multiple  neuritis,  perfectly  charac- 
teristic, came  on  without  any  discoverable  cause,  and  ran  a  course  in 
most  cases  mild,  and  ending  in  recovery ;  in  one  or  two  others  severe, 
and  ending  in  death.  Some  cases  of  what  is  popularly  termed  "  blood- 
poisoning"  are  probably  of  this  nature.  A  condition  also  occurs 
during  the  puerperium,  and  is  probably  the  result  of  some  accidental 
septicaemic  process.  The  type  is  one  of  severe  polyneuritis.*  A 
similar  condition  has  also  been  describedf  as  occurring  during  preg- 
nancy, the  symptoms  all  disappearing  after  delivery.  If  certain  drain 
poisons,  probably  organismal,  are  capable  of  giving  rise  to  a  disease, 
diphtheria,  of  which  multiple  neuritis  is  often  part,  there  is  no  im- 
probability in  some  forms  of  virus,  of  similar  character  and  analogous 
source,  having  such  neuritis  for  their  chief  effect. 

In  this  connection  it  is  instructive  to  note  that  an  epidemic  is  on 
record  in  which  paralysis  preceded  the  throat  affection  (see  Diph- 
theritic Paralysis,  Yol.  II).  As  an  instance  of  this  primary  form  may 
be  mentioned  the  case  of  a  medical  practitioner  who  was  attacked  with 
obstinate  vomiting  and  diarrhoea  succeeded  by  constipation,  for  which 
no  cause  could  be  discovered,  quickly  followed  by  "  numbness  "  in  the 
hands  and  feet,  and  a  difficulty  in  using  them.  When  I  saw  him,  a 
fortniglit  later,  there  was  loss  of  tactile  sensibility,  limited  to  the 
palms  and  soles,  weakness  of  the  legs,  and  no  knee-jerk.  The  weakness 
rapidly  increased  and  extended,  and  at  the  end  of  five  weeks  from  the 
onset  he  died.  Such  a  case  presents  all  the  features  of  the  toxic 
form.  J 

The  Bheumatic  form  embraces  aU  the  cases  of  polyneuritis  that  are 
due  to  exposure  to  cold.  Rheiimatic  fever,  due  to  a  similar  exposure 
to  cold,  must  depend  on  a  morbid  blood- state,  produced  in  some  way 
by  the  disturbing  influence  of  the  chill  on  the  metabolic  processes 
within  the  body.     The  poison  thus  generated  must  vary  much  in  its 

*  Mobius,  •  Munch,  med.  Woclienschr.,*  1892,  Bd.  xlv;  Bernhardt, '  Deut~c-h.  med 
Wochenschr.,*  1894,  Bd.  1;  Eulenburg,  '  Deutsch.  med.  Wocheiischr.,'  1895,  Bd. 
viii,  ix ;  Sottas  and  Sottas,  •  Gaz.  des  Hop.,'  1892;  Turney,  'St.  Thomas's  Hosp. 
Reports,'  vol.  xxv,  1898,  who  describes  three  varieties:  (1)  generalised  neuritis, 
(2)  localised  neuritis  aflecting  (a)  upper,  (J)  lower  limbs,  and  (3)  neuritis  affecting 
a  single  nerve  without  any  ascertain ;ible  local  cause. 

t  Stiefel,  'Neur.  Cent.,'  1893,  352. 

J  It  should  be  mentioned  tliat  other  circumstances  excited  a  suspicion  of  inten- 
tional chronic  poisoning.  This  fact  does  not  lessen  the  suggestiveness  of  the  case. 
Multiple  neuritis  must  henceforth  constitute  an  element  in  many  questions  of  medical 
jurisprudence.  Its  discovery  might  give  greater  weight  to  indications  of  some  forms 
of  poisoning. 


■150  MULTIPLE    NEURITIS. 

precise  nature  to  cause  tlie  great  variations  in  tlie  acute  lesions  wLich 
follow  (see  Chorea,  Yol.  II).  Hence  it  is  not  surprising  that  the 
effect  of  the  exposure  should,  in  some  persons,  be  such  as  to  cause  a 
blood-state  capable  of  producing  multiple  neu:;;tis,  symmetrical  and 
parenchymatous.  In  a  case  recorded  by  Putnam,  fatal  in  seven  days 
from  paralysis  of  the  respiratory  muscles,  following  directly  exposure 
to  cold,  there  was  swelling  of  the  spleen  such  as  is  met  with  in  typhoid 
and  other  acute  toxsemic  states.  Two  cases  of  fatal  septic  polyneuritis 
accompanied  by  endocarditis  are  recorded.*  We  do  not  know  to 
what  these  variations  are  due.  In  some  there  has  been  a  previous 
exposure  to  chronic  alcoholism  sufficient  to  explain  the  extreme 
degree  of  the  resulting  process.  The  causes  of  the  differences  in  the 
results  are,  however,  still  involved  in  mystery.  From  this,  as  from 
other  causes,  but  perhaps  with  greater  frequency,  the  blood-state  is 
apt  to  give  rise  to  more  irregTilar  central  inflammation,  and  the  two  may 
coincide.  The  connective  tissue  of  nerves  is,  moreover,  of  the  same 
natui'e  as  that  on  which  the  rheumatic  poison  exerts  its  special  influence, 
and  the  modes  of  onset  of  sciatica  have  shown  us  that  the  rheumatic 
process  may  pass  from  the  fasciae  to  the  nerve-sheaths.  A  considera- 
tion of  the  facts  makes  it  probable  that  cold  may  cause  (1)  an 
isolated  adventitial  neuritis ;  (2)  a  multiple  neuritis  of  the  same 
character,  irregular  in  distribution,  probably  by  the  agency  of  a  blood- 
poison  related  to  that  which  causes  muscular  rheumatism  and  the 
affection  of  the  fibrous  tissues  of  the  joints  in  rheumatic  fever ;  (3) 
a  symmetrical  parenchymatous  polyneuritis  due  probably  to  a  special 
blood-state  of  greater  specificity  than  that  which  produces  the 
adventitial  form,  and  perhaps  allied  to  that  which  gives  rise  to 
pneumonia  and  to  cerebro-spinal  meningitis.  Pneumonia,  at  least, 
may  be  combined  with  multiple  neuritis  as  a  result  of  the  same 
exposure. 

The  diabetic  form  presents  many  varieties,  and  our  knowledge  of 
it  has  been  already  referred  to  so  far  as  its  imperfect  character 
permits.  It  is  not  related  to  the  amount  of  sugar  in  the  urine — 
in  many  of  the  sufferers  this  has  been  small  (in  one,  indeed,  as 
low  as  one  hcJf  per  cent.)  ;  nor  is  it  readily  influenced  by  a  dietetic 
reduction  of  the  amount  of  sugar  in  the  blood.  Hence,  as  we  have 
seen,  it  is  supposed  to  be  due  to  some  toxic  product  that  is  i<  rmed  in 
the  perverted  metabolism  of  the  diabetic  subject,  comparable  to  acetone, 
although  certainly  not  acetone  itself  .f 

Alcoholic  polyneuritis  is  the  form  most  frequently  met  with,  at  anv 
rate  so  far  as  well-marked  cases  are  concerned ;  this  form  preponderates 
in  most  countries  over  all  others  put  together,  but  at  present  many  of 
the  slighter  sensory  forms  escape  recognition,  and  the  preponderance 

*  Lloyd  and  Riesman,  *  Trans.  Am.  Neur.  Assoc.,'  1892. 

f  Williamson  ('Med.  Cliron.,'  Nov.,  1892)  lound  in  fifty  cases  of  diabetes 
that  the  knee-jerk  was  absent  in  38  per  cent,  and  obtained  only  with  reinforcements 
in  12  per  cent. 


ETIOLOGY.  151 

will  probably  be  much  less  when  all  cases  are  recognised.  It  results 
chiefly  from  the  stronger  forms  of  alcohol,  especially  from  spirits.  It 
is  more  common  among  those  who  take  small  quantities  frequently 
than  among  those  who  indulge  in  an  occasional  "  bout "  of  intem.- 
perance,  probably  because  the  total  quantity  consumed  is  greater. 
Strange  to  say,  it  is  far  more  frequent  among  women  than  among  men, 
probably  at  least  three  times  as  frequent.  The  cause  for  this  is  difficult 
to  discern.  On  the  one  hand  continuous  drinking  is  more  common 
among  women  than  is  occasional  intoxication ;  and  women  who  take 
alcohol  to  excess  generally  do  so  in  the  form  of  spirits,  while  men 
often  take  it  in  the  form  of  beer.  At  the  same  time  the  opposite  pro- 
clivity is  seen  in  the  case  of  delirium  tremens,  and  it  would  seem  as  if 
there  were  a  difference  in  the  nervous  system  of  the  two  sexes,  whereby 
a  susceptibility  of  the  nerves  in  women  replaces  that  of  the  brain  in 
men. 

The  amount  of  alcohol  habitually  taken,  and  the  duration  of  the 
habit  before  the  onset  of  the  affection,  vary  much.  It  has  been  thought 
that  a  neuropathic  disposition  renders  a  less  quantity  and  briefer 
period  adequate.  Other  causes  often  co-operate  with  alcohol  in 
exciting  the  disease,  especially  exposure  to  cold,  and,  in  the  lower 
classes,  insufficient  nourishment.  Exposure  is  especially  effective  in 
those  in  whom  slight  symptoms  exist  but  attract  little  attention,  and 
the  condition  of  the  nervous  system  is,  as  it  were,  prepared  for  a 
severe  outbreak. 

Idiopathic  Forms. — Regarding  these  varieties  of  idiopathic  poly- 
neuritis, little  remains  to  be  added  to  what  has  been  stated  at  p.  146. 
The  evidence  rests  chiefly  on  the  post-mortem  discovery  of  the  changes 
in  the  nerves,  but  it  is  probable  that  more  careful  attention  to  the  point 
win  show  that  symptoms  are  not  unfrequent  which  now  receive  Kttle 
attention,  or  are  regarded  as  functional  only.  More  attention  is  also 
needed  by  the  cases  in  which  a  neuropathic  tendency  or  some  depressing 
emotion  seems  to  be  the  cause  of  this  disease,  as  they  may  be  of  the 
corresponding  degeneration  of  the  pyramidal  fibres  within  the  cord  in 
"lateral  sclerosis,"  a  degeneration  which  also  begins  at  the  extremity 
of  the  fibres  and  extends  upwards. 

Atheromatous  Neuritis  merits  separate  recognition  on  account  of  ita 
peculiar  mechanism.  In  these  cases  the  nerves  of  the  limbs  have  been 
extensively  damaged  through  atheroma  (or  "  arteritis  obliterans  ")  of 
their  arteries.  Necrotic  inflammatory  processes  take  place  in  the  parts 
of  the  nerves  supplied  by  the  affected  vessels,,  and  there  result  symptoms 
that  resemble  those  of  the  toxic  form  in  character,  although  differing 
in  the  greater  irregularity  of  distribution.  The  symmetry  that  consti- 
tutes so  striking  a  feature  of  the  common  cases  is  absent,  but  arterial 
degeneration  may  be  symmetrical,  and  an  incomplete  rough  symmetry- 
may  thus  result.  It  is  conjectured  that  such  disease  of  the  nerves  will 
be  found  to  be  frequent  in  the  cases  in  which  senile  disease  of  the 
arteries  gives  rise  to  gangrene,  and  that  it   is   partly   through  the 


152  MULTIPLE    NEURITIS. 

Qigencj  of  the  nem-itis  that  the  gangrene  is  produced ;  but  tMs  vie"W 
overlooks  llie  potent  influence  of  the  arterial  obstruction  itself.* 

General  Etiology. — Multiple  neuritis  is  a  disease  of  adult  life. 
The  only  form  met  with  in  children  is  that  which  sometimes  accom- 
panies polio-myelitis,  and  may  perhaps  now  and  then  be  met  with 
apart  from  the  spinal  malady,  as  an  infantile  variety  of  multiple 
adv-entitial  neuritis,  irregular  in  distribution.  The  other  common 
f onus  occur  chiefly  between  twenty  and  fifty ;  the  alcoholic  between 
thirty  and  forty  twice  as  frequently  as  in  either  the  previous  or  later 
decade,  but  it  may  be  met  with  up  to  sixty  years  of  age ;  while  the  rare 
senile  (purely  degenerative)  variety,  and  the  still  more  rare  arteritic 
form,  are  met  with  at  still  later  ages.  Females  constitute  a  majority 
of  the  cases,  the  degree  of  which  cannot  yet  be  estimated;  it  is  due 
solely  to  their  liability  to  alcoholic  neuritis,  in  which  they  amount  to 
at  least  70  per  cent.  The  rheumatic  and  toxaemic  forms,  on  the  other 
hand,  occur  more  frequently  in  males,  probably  from  their  greater 
exposure  to  the  causes  of  the  disease. 

More  than  one  cause  may  co-operate  in  producing  the  affection,  and 
then  we  may  distinguish  the  one  as  "  predisposing,"  the  other  as 
"  exciting,"  although,  as  a  rule,  either  would  be  adequate  alone  were 
its  degree  more  intense.  The  double  causation  can  most  often  be 
traced  when  cold  or  some  depressing  influence  co-operates  with  alcohol, 
or  when  the  influence  of  the  latter  is  conjoined  with  that  of  tubercle, 
as  either  the  primary  or  secondary  cause.  Even  more  frequently  the 
symptoms  follow  some  cause  of  constitutional  depression,  inadequate 
alone.  In  one  case  this  was  an  attack  of  acute  gastric  catarrh  with 
vomiting,  the  result  of  the  alcoholism  which  was  the  real  cause  of  the 
neuritis.  In  other  cases  a  depressing  emotion  has  seemed  to  be  the 
excitant,  and,  as  we  have  seen,  this  appears  to  be  sometimes  alone 
sufficient.  In  one  alcoholic  case  the  injurious  effect  of  painful  emo- 
tion (due  to  the  death  of  a  son)  was  at  once  to  arrest  improvement, 
and  to  excite  a  progressive  increase  in  the  symptoms,  which  continued 
until  death  a  few  months  later.  Ansemia  probably  acts  chiefly  as  a 
predisponent,  lowering  the  nutrition  and  resisting  power  of  all  the 
tissues,  and  rendering  a  slighter  cause  effective.  Thus,  in  the  case  of 
an  anaemic  girl  recorded  by  Barrs,  the  symptoms  followed  immediately 
on  bathing  the  feet  in  cold  water,  when  the  patient  was  heated. 

Symptoms. — The  symptoms  produced  by  multiple  neuritis  differ 
widely,  as  has  been  already  intimated,  according  to  the  character  of 
the  affection  of  the  nerves.  They  are  of  three  classes — motor  weak- 
ness, sensory  distui-bance,  and  inco-ordination.  Psychical  symptoms 
are  sometimes  pronounced,  especially  in  alcoholic  cases.  Loss  of 
memory,  especially  as  regards  place  and  time,  are  the  most  obvious 
conditions,  but  hallucinations,  especially  visual,  are  not  infrequently 

*  Schlesinger  (■  Neur.  Cent.,'  1895,  p.  578)  describes  a  form  of  neuritis  associated 
with  endarteritis  obliterans  similar  to  tliat  occurring  in  syphilis.  . 


SYMPTOMS, 


153 


present.  The  motor  weakness  depends  on  an  affection  of  the  motor 
nerve-fibres,  and  usually  involves  first  and  cliiefly  the  flexors  of  the 
ankle  and  extensors  of  the  toes,  and  the  extensors  of  the  wrist  and 
fingers  in  the  forearm,  muscles  that  are  homologous  and  supplied  by 
corresponding  nerves,  the  anterior  tibial  or  peroneal  nerve  in  the  leg,  the 
radial  branch  of  the  musculo-spiral  in  the  arm.  The  result  is  the  cha- 
racteristic "wrist-drop"  and  "foot-drop,"  shown  in  Fig.  58.      Other 


FiGt.  58. — Multiple  alcoliolic  neuritis;  palsy  of'  extensors  of  wrist  and  flexors 
of  ankle.     (Prom  a  photograph  by  Mr.  Hyde  Marriott,  B.Sc.) 


muscles  suffer  in  severe  cases.  The  sensory  symptoms  consist  of  sub- 
jective sensations  of  tingling,  &c. ;  pains  of  various  character,  seat, 
and  degree,  often  referred  to  the  nerves  or  deep  parts ;  tenderness 
of  the  skin,  nerve-trunks,  and  muscles,  and  loss  of  cutaneous  sensi- 
bility. The  inco-ordination  resembles  that  present  in  the  slighter 
degrees  of  locomotor  ataxy,  and  is  often  associated  with  loss  of  the 
muscular  sense.  These  symptoms  will  be  considered  in  detail.  Ac- 
cordmg  to  the  predominance  of  one  or  other  of  the  three  sets, 
corresponding  varieties  are  distinguished, — (1)  a  motor  form,  in 
which  there  is  loss  of  power  with  or  without  the  other  syrrr^toms; 
(2)  a  sensory  form,  in  which  there  is  no  marked  muscular  \-^.„Auess 
or  inco-ordmation,  but  in  which  sensory  disturbance — irritation  or 
loss — is  the  chief  symptom;  (3)  an  ataxic  form,  in  which,  without 
marked  loss  of  power,  with  or  without  sensory  symptoms,  inco-ordina- 
tion is  the  dominant  symptom. 

As  mentioned  m  the  introductory  section,  the  varieties  of  poly- 
neuritis that  are  due  to  special  metallic  poisons  and  to  certain  acute 
specific  diseases  are  described  separately,  in  the  second  volume  of  this 
work,  among  the  general  diseases  of  the  nervous  system.  The 
common  alcoholic  form  is  alone  suitable  to  convey  a  knowledge  of  the 
general  features  of  the  affection,  but  the  account  of  its  symptoms 
must  be  completed  by  some  mention  of  those  of  the  less  common 
forms,  which  do  not  need  separate  description  elsewhere. 

It  should  1  le  clearly  understood  at  the  outset  that  no  set  of  sym- 
ptoms is  exclusively  related  to  a  single  cause.     The  various  forms 


154  MULTIPLK    NKUi;nTS. 

which  depend  on  the  incidence  of  the  disease  on  the  motor  or  the 
sensory  nerves  in  preponderant  degree,  are  met  with  in  consequence 
of  each  of  the  common  causes,  although,  as  we  shall  see,  some  causes 
give  rise  more  frequently  to  one  form  than  to  another. 

The  onset  and  course  of  multiple  neuritis  vary  much  in  different 
cases,  both  of  the  same  class  and  of  the  several  classes.  Premonitory 
symptoms  occur  in  some  cases.  They  have  been  observed  chiefly  in 
the  alcoholic  form,  but  occur  in  others  also.  When  due  to  cold  or 
3ther  causes  of  constitutional  disturbance,  the  special  symptoms  of 
the  general  state  may  immediately  precede  the  onset.  Thus,  after 
exposure  to  cold,  general  rheumatic  symptoms,  with  fever,  bron- 
chitis, and  other  signs  of  catarrh,  may  immediately  be  followed  by 
the  symptoms  of  neuritis.  The  symptoms  that  have  been  regarded  as 
premonitory  are  especially  "  numbness  "  and  tingling  of  the  fingers 
and  toes,  or  of  the  palms  and  soles,  or  the  lower  parts  of  the  limbs, 
vaso-motor  disturbance  in  the  extremities  (as  pallor  of  the  fingers), 
and  painful  cramp  in  the  calves  or  elsewhere,  together  with  dull 
rheumatoid  pains.  These  constitute  the  first  symptoms  of  the  disease, 
but  when  they  exist  alone  for  months  before  the  onset,  they  may 
be  regarded  as  "premonitory."  All  these  symptoms  are  probably 
due  to  the  influence  of  alcohol  on  the  nerves,  disturbing  the  function 
of  the  structures  whose  nutrition  undergoes  subsequently  a  corre- 
sponding impairment. 

The  onset  itself  may  be  acute  or  sub  chronic,  and  is  sometimes  so  slow 
as  to  deserve  the  name  of  chronic,  especially  in  the  slighter  sensory 
form.  An  acute  onset  is  on  the  whole  rare  in  alcoholic  cases  unless 
the  malady  is  "  excited  "  by  some  cooperant  influence.  Thus,  in  one 
alcoholic,  a  prostrating  attack  of  vomiting,  lasting  for  ten  days,  was 
followed  by  an  onset  of  the  symptoms  of  neuritis  so  acute  that  at  the 
end  of  a  week  the  patient  was  unable  to  stand,  and  there  was  general 
impairment  of  sensibility.  It  is  met  with  chiefly  in  cases  due  to 
cold  or  toxsemic  states  ;  in  these  it  may  be  attended  by  severe  con- 
stitutional disturbance,  rigors,  and  considerable  pyrexia.  In  other 
forms  the  elevation  of  temperature  is  usually  slight  or  moderate,  but 
sometimes  amounts  to  3^  or  4°,  and  may  last  from  one  to  three  or 
four  weeks.  The  first  definite  symptom  (if  it  has  not  existed  before)  is 
often  the  tingling,  or  "  pins  and  needles  "  in  the  extremities,  already 
mentioned,  with  vague  "  rheumatic  "  pains,  which  become  more  acute. 
Motor  symptoms  are  usually  soon  added,  palsy  or  inco- ordination, 
or  both.  They  are  seldom  absent  in  acute  cases.  The  pronounced 
symptoms  of  the  disease  may  resemble  in  character  those  produced 
by  the  inflammation  of  single  nerves,  differing  only  in  distribution, 
and  involving  all  the  functions  of  the  affected  nerves  ;  this  is  often 
the  case  when  the  malady  is  acute  in  onset.  But  when  chronic, 
as  already  stated,  the  fibres  may  be  aftected  according  to  their 
specific  function,  and  to  this  the  symptoms  are  then  limited,  so  that 
they   resemble  those  that  are  ascribed   to   a  distn.i  1  )ar.ce  of  special 


SYMPTOMS.  155 

elements  of  the  spinal  cord,  rather  than  those  of  an  affection  of 
isolated  nerves. 

The  motor  form  involves  either  the  upper  or  lower  limbs  or  both, 
but  always  the  corresponding  limbs  on  both  sides,  and  their  distal 
extremities,  the  hands  or  the  feet,  first  and  chiefly.*  Whichever 
limbs,  arms  or  legs,  present  the  first  symptoms,  suffer  in  greater 
degree  throughout,  and  it  may  be  alone.  The  feet  suffer  more  fre- 
quently than  the  hands  ;  motor  symptoms  may  exist  in  the  legs,  and 
only  sensory  symptoms  in  the  hands,  and  this  when  the  weakness  in 
the  legs  is  extreme.  In  whatever  part  the  affection  occurs,  various 
sensory  disturbances,  and  especially  pains,  commonly  precede  the 
weakness,  and  increase  with  it.  They  are  accompanied  by  tenderness, 
generally  along  the  nerve-trunks,  but  almost  invariably  in  the  mus- 
cles, and  sometimes  of  the  skin,  These  pains  may  occur  in  the 
limbs  that  do  not  become  paralysed  if  the  action  of  the  cause  can 
be  arrested.  Although  loss  of  power  is  the  obtrusive  feature,  some 
loss  of  co-ordination  generally  accompanies  it,  and  is  often  the  means 
by  which  the  attention  of  the  patient  is  first  directed  to  the  com- 
mencing disability.  lie  finds  a  diflficulty  in  balancing  when  standing, 
or  in  performing  the  finer  movements  with  his  fingers;  and  his 
attention  being  thus  drawn  to  the  part,  he  finds,  on  further  investiga- 
tion, that  there  is  some  actual  defect  in  the  power  of  extending  the 
wrist  and  fingers,  or  in  raising  the  toes  or  foot  from  the  ground  during 
the  forward  movement  of  the  foot  in  walking.  Tremor  is  often  con- 
spicuous in  alcoholic  cases  long  before  the  loss  of  power.  Whenever 
there  is  weakness  in  the  leg  the  knee-jerk  can  no  longer  be  obtained, 
probably  because  the  afferent  muscle-nerves  sufl'er  more  widely  than 
the  motor  nerves.  The  constancy  of  this  loss  is  very  great,  and  it  is 
not  related  to  any  one  class  of  symptoms,  but  occurs  equally  in  the 
paralytic  and  ataxic  forms  of  neuritis.  At  the  same  time  it  is  not 
absolute ;  the  knee-jerk  seems  to  persist  in  very  rare  cases  of  slight 
degree,  as  mentioned  on  p.  160. 

"When  the  first  symptoms  are  in  the  legs,  the  first  motor  loss  is 
the  inability  to  raise  the  toes  from  the  ground  in  walking,  owing  to 
impei-fect  flexion  of  the  ankle-joint  and  extension  of  the  toes.  But 
this  symptom  may  be  to  a  large  extent  obscured  by  the  interference 
v/ith  movement  occasioned  by  the  tenderness  of  the  muscles  and  the 
hyptTBestliesia  of  the  skin,  which  commonly  coexist  and  prevent  the 
exertion  of  the  amount  of  power  that  is  possessed.  There  is  a 
tendency  for  the  nerves  of  the  sole  and  the  palm  of  the  hand  to  be 
especially  involved  in  the  hyperaesthesia — ^in  obedience  to  a  law  of 
pathological  susceptibiUty  which  is  to  be  traced  not  infrequently  in 
polyneuritis,  especially  in  the  tabetic,  diphtheritic,  and  toxsemic  forms. 

These  effects  of  the  sensory  irritation  may  render  it  difficult  to  deter- 
mine the  common  course  of  the  motor  weakness,  the  order  in  which  the 

*  The  very  vai-inu-  symptoms  of  the  disease  arc  well  illnstrHted  by  an  instructive 
series  of  cases  pulilisliod  by  Dr.  Buzzard. '  Par;dvsis  from  Peripheral  Neuritis,'  1886. 


156  MULTIPLE    NEURITIS. 

muscles  suffer,  and  the  relative  degree  of  their  impairment  at  different 
periods.  The  conspicuous  fact  is  that  the  muscles  first  to  suffer  are 
those  supplied  from  the  external  popliteal  nerve  (anterior  tibial  group), 
and  these  are  followed  by  other  muscles  below  the  knee,  except  in  the 
very  rare  instances  in  which  palsy  does  not  spread  beyond  the  muscles 
first  affected.  The  muscles  above  the  knee  suffer  later  and  less,  and 
more  frequently  escape  considerable  impairment.  The  flexors  of  the 
knee,  although  supplied  by  the  sciatic  nerve,  do  not  suffer  more  than 
the  extensors,  or  more  frequently,  so  far  as  the  tenderness  permits  us 
to  judge.  Least  frequently  and  in  least  degree  the  muscles  suffer  that 
move  the  hip-joint ;  it  is  only  in  the  most  severe  cases  that  these  are 
appreciably  affected.  Very  rarely  is  the  innervation  of  the  bladder  or 
rectum  involved  in  the  disturbance  of  polyneuritis.  Interference  with 
these  functions  may  generally  be  regarded  as  evidence  that  the  morbid 
changes  are  not  limited  to  the  nerves,  but  involve  the  spinal  cord 
itself. 

In  the  arms,  the  first  and  chief  defect  in  power  is  in  the  extensors 
of  the  wrist  and  fingers.  Both  arms  are  affected  together  or  in  quick 
succession— one  never  suffers  alone  in  considerable  degree.  Thus 
the  palsy  resembles  at  first,  and  sometimes  throughout,  that  which  is 
familiar  as  the  effect  of  lead  poisoniag.  The  resemblance  may  extend 
to  the  escape  of  the  extensor  of  the  metacarpal  bone  of  the  thumb, 
and  of  the  supinator  lougus.  Lead  palsy,  indeed,  is  an  example  of 
peripheral  neuritis  of  the  "toxic  form,"  and  differs  from  the  general 
form  in  the  limitation  of  even  severe  palsy.  It  seems  that  the  radial 
nerve  (of  the  musculo -spiral)  is  that  which,  in  the  upper  limbs,  is 
the  most  prone  to  suffer — a  fact  which  is  at  present  unexplained.  It 
probably  depends  not  on  any  peculiarity  of  position,  but  on  some 
deeper  functional  relation  and  position  among  the  nerves  of  the  body, 
since  it  obtains  when  the  affection  is  of  the  peripheral  extremities  of 
the  fibres  and  does  not  involve  all  the  fibres  of  the  nerve.  The  radial 
nerve  is  homologous  with  that  in  the  leg  of  which  the  fibres  are  the 
first  to  suffer — the  peroneal, — regarding  which  the  same  facts  are  true. 

It  is  only  in  the  cases  due  to  metallic  poisons  that  the  palsy  becomes 
considerable  without  spreading  to  other  muscles.  Generally  the  flexors 
of  the  wrist  and  fingers  become  weak,  and  so  do  the  interossei,  while 
the  last  to  suffer  are  the  thenar  and  hypothenar  muscles.  It  is  rare 
for  the  palsy  of  any  muscles  except  the  extensors  to  be  complete.  In 
more  severe  degree  the  muscles  above  the  elbow  suffer,  last  and  least 
those  of  the  shoulder.  The  muscles  below  the  elbow  may  be  abnost 
powerless  when  those  above  it  are  scarcely  affected. 

In  severe  cases  the  trunk  muscles  are  involved — the  diaphragm, 
and  the  muscles  of  the  thorax  and  abdomen.  Less  rarely,  but  also 
only  in  severe  fonns,  increased  frequency  of  the  pulse  and  palsy  of 
the  vocal  cords  indicate  that  the  fibres  of  the  vagus  are  suffering. 
Yeiy  seldom  the  facial  muscles,  or  those  of  the  tongue,  are  involved. 
Affection  of  the  motor  ocular  nerves  and  of  the  pupils  has  been  met 


SYMPTOMS.  157 

with  only  in  the  most  severe  cases,  and  chiefly  in  those  due  to  toxaemia. 
Slight  nystagmus  is  common.  The  nerves  of  the  pharynx  and  ahmen- 
tary  viscera  seem  always  to  escape.  It  is  more  doubtful  whether  this 
is  true  of  the  branches  to  the  lung. 

Thus  the  stress  of  the  affection  falls  on  the  muscles  of  the  extremities, 
and  those  that  are  affected  first  also  suffer  alone  in  slight  cases,  and 
most  in  those  that  are  severe.  As  they  become  weak,  and  sometimes 
even  earlier,  they  become  tender,  and  this  muscular  tenderness  usually 
increases  to  an  extreme  degree,  and  becomes  even  more  obtrusive,  in 
many  cases,  than  the  tenderness  of  the  nerves.  The  limb  cannot 
be  grasped,  even  gently,  without  cries  of  pain  being  elicited,  and 
every  change  of  posture  gives  rise  to  distress.  This  is  an  important 
sign,  and  is  no  doubt  due  to  the  fact  that  aU  the  nerves  of  the  muscle 
suffer,  the  afferent  as  well  as  the  motor  twigs.  Very  rarely  it  is 
absent.  It  increases  the  pain  of  an  electrical  examination,  and,  after 
a  strong  current  has  been  applied,  the  muscles  may  ache  for  hours — 
a  fact  that  should  inculcate  scrupulous  consideration  in  all  such  pro- 
cedures. 

The  affected  muscles  quickly  become  flabby  and  waste,  and  their 
prominences  disappear.  The  muscular  atrophy  becomes  extreme  in 
some  instances,  but  the  aspect  of  the  limbs  differs  from  that  in  pro- 
gressive muscular  atrophy  on  account  of  the  partial  distribution  of  the 
affection,  and  because  it  is  only  in  a  few  muscles,  such  as  the  extensors 
of  the  fingers  and  the  interossei,  that  the  wasting  is  as  great  as  in 
that  affection.  Changes  in  the  electrical  excitability  of  the  muscles 
concur  with  these  symptoms ;  they  present  the  reaction  characteristic 
of  nerve  lesions,  described  at  p.  72.  The  faradic  irritability  is 
lost,  and  that  to  voltaism  is  increased  in  amount,  and  often  but  not 
always  altered  in  quality,  in  the  usual  manner.  In  slight  cases, 
however,  increase  in  voltaic  irritability  may  be  trifling,  and  may  be  the 
chief  change.  In  the  nerves  the  irritability  to  both  currents  lessens 
and  is  ultimately  lost.  If  a  case  is  examined  in  the  very  early  stage, 
the  increased  irritability  mentioned  at  p.  76  may  be  found,  and  after- 
wards replaced  by  diminution  and  loss.  In  the  most  severe  cases  the 
muscles  most  affected  may  quickly  lose  all  irritabihty,  on  account  of 
the  intensity  of  the  secondary  changes  in  the  muscular  tissue,  which 
destroys  instead  of  merely  changing  their  special  structure  and  charac- 
teristic functions.  Occasionally  the  wasting  is  concealed  by  oedema, 
but  now  and  then  the  size  of  the  muscles  may  be  little  reduced  even 
when  they  are  totally  paralysed,  with  extinction  of  faradic  and  great 
reduction  of  voltaic  irritability.  But  the  wasting  is  only  deferred,  and 
extreme  atrophy  ultimately  occurs.  In  such  cases  there  is  probably  a 
rapid  fatty  degeneration  of  the  muscles,  and  the  fat  accumulates  be:  ■we  -n 
the  fibres  so  as  to  maintain,  for  a  time,  the  bulk  of  the  muscle.  An 
extremely  rare  alteration  is  the  preservation  of  some  voltaic  irritability 
in  the  nerves  as  well  as  in  the  muscles,  faradic  irritability  being  lost  in 
both. 


158  MULTIPLE    NETJEITI8. 

As  in  other  cases  of  unequal  palsy,  the  less  affected  muscles  of  the 
limbs  are  apt  to  undergo  contracture  and  shortening,  especially  when 
the  palsy  causes  the  part  to  assume  habitually  a  certain  position 
under  the  influence  of  gravitation,  or  a  certain  posture  is  adopted 
by  the  patient  in  order  to  lessen  pain.  These  contractures  occur 
chiefly  in  the  lower  limbs.  The  foot-drop  shown  in  Pig.  58  is  especially 
frequent,  from  the  palsy  of  the  flexors  permitting  the  foot  to  fall  as 
the  patient  lies  in  bed,  under  the  influence  of  gravitation  and  of  the 
unopposed  extensors.  The  effect  of  posture  is  seen  in  the  contraction 
of  the  flexors  of  che  knee,  which  may  be  fixed  at  a  right  angle,  and  to 
a  less  degree  of  those  of  the  hip,  on  account  of  habitual  flexion  of 
these  joints  to  escape  the  pain  occasioned  when  the  parts  are  put  on 
the  stretch  in  the  extension  of  the  limb. 

The  affection  of  the  sensory  nerres  of  the  skin  is  sometimes  absent; 
more  often  it  is  conjoined  with  that  of  the  motor  nerves,  and  some- 
times only  with  that  of  the  sensory  muscle-nerves.  It  very  seldom 
exists  alone.  Its  first  manifestation,  is  the  tingling  and  other  sub- 
jective sensations  already  mentioned,  to  which  is  soon  added  the 
hyperaesthesia  which  is  often  so  troublesome  throughout  the  disease. 
Sensory  disturbance  is  usually  more  extensive  than  the  motor  palsy, 
but  is  greatest  at  the  most  affected  parts,  the  extremities  of  the  limbs. 
Except  in  slight  cases,  the  damage  to  the  sensory  fibres  becomes  suffi- 
cient to  lessen  the  sensibility  of  the  skin  to  touch.  A  faint  touch 
may  be  unfelt  while  over-sensitiveness  to  pain  continues,  and  is  even 
so  intense  that  a  strong  touch  may  cause  distress.  Rarely,  as  I  have 
twice  seen,  sensibility  to  pain  is  lost,  that  to  touch  being  preserved. 
The  temperature  sense  is  usually  normal.  These  sensory  changes  are 
first  developed  on  the  extremities,  sometimes  on  the  palms  and  soles ; 
they  may  even  be  still  more  local,  and  only  considerable  on  the  tips 
of  the  fingers,  or  the  hyperaesthesia  may  be  chiefly  under  the  nails. 
Spontaneous  pains  accompany  the  hyperaesthesia,  dull,  or  acute,  or 
"burning"  in  character,  referred  to  the  deeper  parts  of  the  limb,  or 
the  nerve-trunks,  or  the  joints,  and  increased  or  excited  by  any 
attempt  at  movement.  The  pain  referred  to  the  joints  may  be  really 
in  them,  especially  in  the  gouty,  or  it  may  be  in  the  nerves  that  pass 
by  the  joints  and  are  disturbed  mechanically  by  their  movement. 
The  sensory  change  increases  in  area  as  the  disease  develops,  and 
usually  extends  first  up  the  outer  side  of  the  leg  and  the  radial  side  of 
the  forearm.  Although  sensibility  to  pain  is  seldom  lost,  the  con- 
duction of  pain  is  delayed  in  some  cases. 

In  the  ataxic  form,  inco-ordination  is  the  chief  symptom,  and  it 
may  accompany  the  onset  of  muscular  weakness  in  the  motor  form. 
It  is  usually  accompanied  by  muscular  tenderness,  and  exists  with  or 
without  the  affection  of  cutaneous  sensibihty  just  described.  It  pro- 
bably depends  on  changes  in  the  afferent  muscle-nerves,  the  impres- 
sions from  which  are  chiefly  concerned  in  the  process  of  co-ordination 
(see  Spinal  Cord,  Functions).      The  implication  of  these  nerves  is 


SYMPTOMS.  159 

shown  by  tlie  muscular  tenderness  that  is  so  marked  a  feature.  The 
inco-ordination  in  these  cases  resembles  closely  that  which  exists  in 
tabes,  and  the  knee-jerk  is  lost  as  in  that  disease,  while  the  frequent 
occurrence  of  pains  in  the  limbs  increases  the  resemblance.  Hence 
the  form  ia  which  this  symptom  is  predominant  has  been  called  pseudo- 
tabes, often  with  the  prefix  alcoholic,  from  the  fact  that  this  is  its  most 
frequent  cause.  It  has,  however,  also  been  met  with  from  arsenical 
poisoning.  The  ataxy  may  involre  the  arms  or  the  legs,  but  is  most 
frequent  in  the  latter.  It  never  reaches  the  degree  met  with  in  advanced 
tabes,  probably  because  very  extensive  and  intense  damage  to  the 
muscle-nerves  only  occurs  when  the  toxic  cause  is  intense ;  its  effect 
then  is  not  limited  to  a  single  set  of  nerves,  but,  involving  the  motor 
also,  produces  a  loss  of  power,  before  which  the  inco-ordination 
ceases  to  be  prominent,  if  it  does  not  of  necessity  disappear.  The 
general  law  that  limitation  needs  chronicity  of  course  and  moderation 
of  degree  holds  good  ia  these  cases  on  the  sensory  as  well  as  on  the 
motor  side.  When  the  inco-ordination  is  the  prominent  symptom  the 
disturbance  of  sensory  nerves  is  slight,  and  if  there  is  extreme 
hyperaesthesia  and  much  loss  of  sensation  there  is  usually  also  motor 
palsy ;  and  thus  this  ataxic  form  is  not  commonly  accompanied  by  the 
intense  sensory  disturbance  of  the  complete  and  characteristic  cases. 
Sensory  loss,  chiefly  to  touch,  is,  however,  occasionally  met  with  in 
chronic  cases  of  the  ataxic  type.  The  resemblance  to  tabes  is  then 
still  greater.  The  distinction  is  considered  in  the  section  on  Dia- 
gnosis. 

Tenderness  of  the  nerve-trunks  in  the  region  of  whose  supply  the 
symptoms  are  greatest  is  a  very  common  symptom,  although  not 
always  readily  recognised,  because  they  are  only  more  tender  than  the 
adjacent  structures,  and  the  degree  of  difference  may  seem  not  great 
when  all  the  parts  are  extremely  sensitive.  It  is  less  frequently  a 
marked  symptom  than  the  tenderness  of  the  muscles,  and  must 
depend  (since  the  pain  is  felt  at  the  spot)  on  the  involvement  of  the 
nerves  of  the  sheath.  It  is  only  in  very  rare  cases,  chiefly  of  irre- 
gular adventitial  neuritis,  that  any  swelliug  of  accessible  nerves  can 
be  distinguished. 

Eeflex  action  from  the  skin  varies  much  in  its  condition.  In  cases 
with  hyperaesthesia  it  is  often  increased,  even  when  there  is  considerable 
motor  palsy ;  the  muscles  that  escape  cause  the  movement,  and  the 
impression  that  reaches  the  reflex  centre  is  doubtless  increased  in 
d(3gree  just  as  is  that  part  of  it  which  reaches  the  brain  and  is  felt 
as  increased  sensation.  But  ia  severe  cases,  with  extensive  loss  of 
power  and  sensation,  the  skin-reflex  may  be  lost.  The  loss  may  be 
observed  to  coincide  with  an  increase  ia  the  other  symptoms,  and 
generally  to  be  related  to  loss  of  sensibnily  to  touch  with  extensive 
motor  palsy ;  it  may,  however,  be  lost  when  sensation  is  perfect.  Occa- 
sionally it  is  lost  out  of  proportion  to  the  other  symptoms,  motor 
or  seubuiy.     The  muscle-reflex  action  (myotatic  initabihty),  as  we 


160  MULTIPLE    NEUEITIS. 

have  already  seen,  is  almost  invariably  lost.  Altliougli  the  knee-jerk 
has  been  observed  to  persist  in  rare  cases  (Dejerine),  its  persistence 
is  quite  exceptional,  and  probably  depends  on  the  escape  of  at  least 
some  of  the  fibres  on  which  the  action  depends.  Possibly,  however, 
the  knee-jerk  is  sometimes  excessive  in  the  early  stages  of  the  disease 
in  consequence  of  an  irritable  state  of  the  nerves  on  which  it  depends, 
similar  to  that  which  gives  rise  to  the  hyperaesthesia.  I  have  more 
than  once  found  it  increased  in  cases  of  slight  sensory  neuritis  (e.  g. 
with  symmetrical  tingling  and  slight  anaesthesia  on  the  soles),  and  in 
one  such  case  it  disappeared  as  the  disease  progressed.  On  the  other 
hand,  it  is  often  lost  when  there  is  scarcely  any  appreciable  weakness 
of  the  extensors,  although  usually  some  tenderness  of  these  muscles 
suggests  an  affection  of  the  afferent  path. 

Trophic  clianges  occur  in  prolonged  cases — in  the  nails,  skin,  and 
hairs,  and  are  similar  to  those  of  ordinary  neuritis ;  glossy  skin, 
arthritic  adhesions,  and  thickening  being  the  most  common.  Bedsores 
are  not  common — a  fact  well  established,  but  not  altogether  easy  to 
explain.  The  vaso-motor  nerves  may  present  disturbance  of  function, 
and  some  oedema  of  the  limbs  is  common,  especially  in  castis  that  are 
due  to  alcohol;  the  blood- state  or  impaired  function  of  tte  kidneys 
probably  aids  in  its  production.  It  occurs  about  the  ankle i^,  back  of 
the  foot,  and  very  frequently  about  the  wrist  and  back  of  the  hand. 
In  one  very  severe  and  fatal  alcoholic  case  the  legs  and  back  were 
the  seat  of  a  peculiar  brawny  oedema,  pitting  very  slowly,  and 
attended  with  lividity  of  the  skin  in  the  depending  parts,  which 
became  brighter  in  colour  on  pressure.  In  this  case  endocarditis 
developed  during  the  course  of  the  malady.  In  one  recorded  case 
Cfidema  was  present  only  over  the  nerve-trunks  in  the  neighbourhood 
of  joints — a  place  in  which  the  movement  of  the  limbs  especially 
disturbs  the  sheath-nerves  of  the  trunks,  to  which  the  vaso-motor 
nerves  of  the  part  are  probably  related.  Indeed,  it  is  common  for 
osdema  to  be  present  about  the  joints  when  it  is  not  elsewhere,  and 
this  may  be  the  explanation.  Pain  in  the  joints  and  even  some 
effusion  into  them  have  been  observed,  especially  in  association  with 
an  acute  onset,  but  it  is  perhaps  more  often  gouty  *  than  rheumatic 
in  alcoholic  cases,  and  its  significance  as  to  the  "  rheumatic  "  nature 
of  the  neuritis  must  be  looked  on  with  some  suspicion.  It  may 
be  occasionally  a  result  of  the  disease  of  the  nerves,  since  chronic 
changes  in  the  joints  often  take  place  just  as  they  do  in  the  inflam- 
mation of  single  nerves.  It  is  especially  common  in  the  finger- joints 
and  in  the  wrist,  but  it  occurs  also  in  the  shoulder  and  knee,  less 
frequently  in  the  elbow,  and  in  the  joints  of  the  leg  other  than  the 
knee.  There  is  pain  on  movement  and  thickening  about  the  joint, 
with  ultimate  limitation  of  movement.  It  is  a  troublesome  effect  of 
the  disease,  because  it  interferes  with  movement  when  power  returns. 

•  Acute  gout,  it  must  be  remembered,  sometimes  attacks  the  larger  joints  with  oj 
before  the  smaller  ones,  and  may  closely  resemble  an  attack  of  rheum.atic  fever. 


SYMPTOMS.  161 

There  is  mticli  more  tendency  to  it  in  some  cases  tlian  in  others,  and  it 
may  be  so  extensive  as  permanently  to  cripple  the  sufferer.*  The 
adhesions  commonly  re-form  after  they  have  been  forcibly  broken 
down. 

The  symptoms  are  wide-spread  in  proportion  to  the  acuteness  and 
intensity  of  the  malady,  but  differ  in  these  respects  also  according 
to  its  cause.  The  effect  of  metaUic  poisons  is  usually  limited  and 
confined  to  the  arms,  as  in  lead  palsy.  In  alcoholism  all  the  limbs 
are  often  affected,  but  the  arms  escape  more  often  than  the  legs. 
From  malaria  the  legs  usually  suffer  alone ;  such  cases  are  considered 
at  p.  185.  In  cases  with  complex  causation  a  general  parenchymatous 
neuritis  may  be  accompanied  by  a  preponderant  adventitial  inflam- 
mation of  some  one  nerve,  as  the  facial  or  sciatic.  The  cases  with 
most  widely  distributed  symptoms  are  those  that  result  from  obscure 
toxsemic  states,  from  cold,  and  from  alcoholism.  It  is  in  these,  and 
especially  in  the  first,  that  the  nerves  of  the  heart,  larynx,  and  of  the 
muscles  of  respiration  most  frequently  suffer.  When  the  laryngeal 
nerves  suffer  it  is  usual  for  both  adduction  and  abduction  of  the 
vocal  cords  to  be  weakened,  seldom  one  movement  only.  The  optic 
nerves  have  been  affected  only  in  rare  cases  with  considerable  blood- 
change.  In  the  case  mentioned  on  p.  160  (in  which  there  was  brawny 
oedema  and  endocarditis)  there  was  slight  optic  neuritis  and  a  diffuse 
cedematous  retinitis.f  The  face  has  also  been  affected  on  both  sides 
in  some  of  the  cases  due  to  exposure  to  cold,  and  also,  usually  in 
shght  degree,  in  some  toxsemic  cases;  it  seems  always  to  escape  in 
cases  due  simply  to  alcohol.  The  sphincters  almost  always  escape; 
a  slight  impairment  of  function  has  indeed  been  met  with  in  a  few 
very  severe  cases  of  probably  pure  polyneuritis,  but  their  affection, 
as  already  stated,  usually  indicates  an  implication  of  the  spinal  cord. 

The  Course  of  the  affection  is  determined  by  the  same  conditions  as 
mfluence  its  intensity,  and  by  the  extent  to  which  the  cause  is  imder 
control.  The  more  severe  and  acute  the  symptoms,  the  wider  is  their 
range  and  the  longer  is  their  diiration.  Very  commonly  the  symptoms 
increase  during  three  to  six  weeks,  then  become  stationary  (if  the  cause 
is  stopped),  and  after  one  or  two  months  slowly  improve.  The  first 
sign  of  improvement  is  a  diminution  in  the  pain  and  hyperaesthesia, 
but  the  tenderness  of  the  muscles  and  the  nerve-pains  on  movement 
often  continue  long  after  the  spontaneous  pains  have  ceased.  If 
tingling,  &c.,  ceased  as  the  disease  advanced,  their  return  may  herald 
improvement.  Power  slowly  returns,  first  in  the  muscles  affected  last 
and  least,  and  afterwards  in  those  paralysed  and  wasted  tn  greater 
degree.  In  these  the  weakness  lasts  for  many  months,  and  shortening 
of  the  opponents  of  the  most  affected  muscles  is  apt  to  occur.  The 
contracture  of  the  caK  muscles,  secondary  to  palsy  of  the  flexors  of  the 
ankle,  constitutes  a  grave  hindrance  to  the  use  of  the  legs  in  standing 

•  As  in  a  case  recorded  by  Taylor,  *  Guy's  Hosp.  Eep.,*  1888. 
t  The  changes  are  described  by  Edmunds  and  Lawford, '  Trans.  Opb-  See.,*  ix,  137. 
VOL.  I.  11 


162  MULTIPLE    NEUniTlS. 

and  "walking.  The  patient  cannot  get  th.e  s6le  and  lieel  on  tlie  ground 
so  as  to  afford  a  uniform  base  of  support,  and  keep  Ms  body  upright 
so  as  to  balance  it.  But  this  contracture  invariably  yields  to  persistent 
efforts  to  walk.  The  palsy  of  the  arms  lasts  longer  than  that  of  the 
legs,  or  vice  versa,  according  as  the  one  or  other  suffers  most.  The 
excess  of  impairment  of  the  muscles  most  affected  is  often  very  con- 
spicuous .during  recovery.  Improvement  goes  on  for  a  long  time, 
generally  until  recovery  is  perfect,  but  very  rarely  there  is  some  per- 
manent loss  of  power.  It  is  remarkable,  however,  how  almost  complete 
a  recovery  may  take  place,  even  after  the  paralysis  has  been  consider- 
able for  a  year.  The  wasting  lessens  and  power  returns ;  latest  where 
there  is  most  loss  of  irritability,  and  usually  later  in  the  small  muscles 
of  the  hand  than  in  the  muscles  of  the  forearm.  Relapses  are  apt  to 
occur,  but  only  when  the  cause  of  the  neuritis  is  still  in  operation  in 
some  degree,  or  when  the  patient  is  again  exposed  to  it  or  to  some 
adjuvant  cause,  such  as  cold. 

But  the  malady  sometimes  runs  a  very  acute  course,  especially  in 
the  cases  due  to  toxaemia  or  to  cold,  so  that  all  parts  of  the  limbs  have 
become  weak  and  the  extremities  powerless  by  the  end  of  seven  or  ten 
days,  and  the  involvement  of  the  nerves  of  the  respiratory  muscles 
may  bring  life  to  an  end  within  a  fortnight,  sometimes  even  within  a 
week.  In  this  form  there  may  be  severe  initial  pain,  not  specially 
related  to  the  regions  afterwards  affected.  In  one  case  it  was  severe  in 
the  back,  in  another  across  the  abdomen.  It  is  probably  a  direct 
effect  of  the  blood- state,  distinct  from  its  influence  on  the  peripheral 
nerves.  Such  cases  bear  considerable  resemblance  to  those  of  "  acute 
ascending  paralysis  "  in  their  course — differing,  however,  in  the  fact 
that  the  asceasion  is  up  the  limbs  rather  than  up  the  general  frame. 
Sometimes,  on  the  other  hand,  the  disease  presents  a  course  far  more 
chronic  than  that  described  above,  and  occupies  many  months  in  its 
development  and  progress.  This  is  often  the  case  with  the  limited 
alcoholic  forms,  especially  the  ataxic  variety,  "  pseudo-tabes,"  and  with 
cases  in  which  the  toxic  agent  is  absorbed  from  without  very  slowly, 
and  its  effect  accumulates  gradually,  as  in  some  cases  of  chronic 
arsenical  poisoning.  Slight  cases,  again,  treated  promptly  have  been 
known  to  recover  in  a  month. 

The  temperature,  usually  raised  in  an  acute  onset  (see  p.  154),  may 
be  normal  in  chronic  cases,  with  or  without  an  occasional  rise,  or  may 
be  sHghtly  raised  during  the  chief  part  of  the  course  of  the  disease. 
A  persistent  considerable  elevation  suggests  some  comphcation,  such 
as  phthisis,  and  should  lead  to  careful  and  repeated  examination  of 
the  lungs.  It  may  also  indicate  a  persistence  of  toxaemia,  other  indi- 
cations of  which  are  mentioned  among  the  complications  of  the  disease. 

The  Cause  of  Death  in  acute  cases  is  often  palsy  of  the  respiratory 
muscles,  sometimes  aided  by  a  catarrhal  bronchitis,  or  paralysis  of  the 
heart  when  the  vagus  is  affected ;  in  more  chronic  cases  it  may  be 
caused  by  exhaustion,  due  to  chronic  gastric  derangement  and  the 


COMPLICATIONS.  163 

wearying  effects  of  prolonged  siiffermg,  or  the  effects  of  disease  of  tlie 
spinal  cord.  Pneumonia  frequently  causes  death  in  the  acute  forms, 
and  in  alcoholic  subjects  the  inflammation  of  the  lung  so  often  runs 
a,  low  course,  with  extensive  consolidation,  sometimes  bilateral  and 
unusual  in  seat  (as  in  the  middle  lobe  and  central  portions  of  the 
lung) ,  that  it  is  difficult  to  avoid  the  suspicion  that  its  form  and  course 
are  determined  by  coincident  neuritis  of  the  pulmonary  branches  of  the 
vagus,  either  primary  or  secondary  to  the  inflammation  of  the  lung. 
Death  may  result  unexpectedly  from  cardiac  failure  when  no  preceding 
symptoms  have  suggested  an  affection  of  the  cardiac  nerves,  and  in 
such  cases  there  is  generally  a  coincident  degeneration  of  the  walls  of 
the  heart.  Fatal  neuritis  of  the  vagus,  however,  may  occur  very  early 
in  relapses.  Other  coincident  effects  of  chronic  alcoholism  are  frequent 
causes  of  a  fatal  issue,  such  as  cirrhosis  of  the  liver,  gastric  troubles, 
and  chronic  cerebral  meningitis  in  cases  of  alcoholism,  or  acetonsemia 
in  cases  of  diabetic  polyneuritis.  Various  morbid  blood-states,  causing 
the  neuritis,  may  continue  and  cause  death.  Lastly,  phthisis  is  a  fre- 
quent cause  of  death,  and  probably  varies  in  the  relation  it  bears  to 
the  neuritis,  sometimes  being  its  consequence,  and  sometimes  appa- 
rently a  coincident  effect  of  some  obscure  cause. 

Complications. — The  chief  of  these  have  been  just  enumerated  in 
the  hst  of  the  causes  of  death.  The  most  important  in  alcoholic  cases 
are  the  effects  of  alcohol  on  various  organs  and  tissues,  especially  on 
the  liver,  heart,  and  spinal  cord.  It  is  very  common  to  find  the  liver 
enlarged,  sometimes  very  large ;  less  commonly  it  is  contracted.  The 
enlarged  hver  may  be  either  fatty  or  fibroid.  Ascites  and  other  effects 
of  portal  obstruction  are  much  less  frequent  complications  than  might 
be  expected,  perhaps  because  it  is  rare  to  have  much  contraction  of  the 
new  tissue  in  the  liver.  The  kidneys  also  are  often  diseased.  Gastric 
catarrh  with  its  varied  symptoms,  morning  vomiting,  anorexia,  and  the 
hke,  is  very  common.  So  also  are  the  several  effects  of  gout  due  to 
the  alcoholism  or  to  metallic  poisons  when  these  have  been  the  cause 
of  the  neuritis  (as  lead,  or,  as  in  one  case  under  my  notice,  silver). 
On  the  part  of  the  nervous  system  there  may  be  various  subacute  and 
chronic  forms  of  inflammation  of  the  substance  of  the  spinal  cord  or  of 
its  membranes,  the  common  manifestations  of  which  are  to  a  large  ex- 
tent prevented  by  the  affection  of  the  nerves.  The  most  important,  the 
ti-unk  girdle-pains  and  the  affection  of  the  sphincters,  are  further  con- 
sidered in  the  section  on  Diagnosis.  Chronic  cerebral  meningitis  is  also 
common,  and  usually  fails  to  produce  its  common  manifestation,  pain, 
even  when  marked  and  extensive  opacity  of  the  membranes  and  increase 
of  fluid  are  foTind  after  death.  It  usually  causes  some  chronic  mental 
disturbance,  often  shght  optic  neuritis,  and  sometimes  general  convtd- 
sions.  But  mental  change  is  also  common  as  a  direct  effect  of  the 
alcohol  on  the  brain ;  distinct  delirium  tremens  often  coincides  with  the 
onset  of  the  neuritis.  In  most  alcohohc  cases,  indeed,  the  mer.tal  state 
is  imnatural ;  the  patient  is  irritable,  intolerant  of  pain,  and  deficient  in 


164  MULTIPLE    NEURITIS. 

self-control.      Yarious  manifestations  of  hysteria  occur  in  women,  and 

a  peculiar  childish  jocularity  is  sufficiently  common  to  deserve  special 
mention.  The  craving  for  withheld  stimulants  increases  the  irritability, 
and,  in  the  case  of  women,  previous  training  in  duplicity  in  order  to 
obtain  alcohol  has  usually  induced  a  general  deterioration  of  the  moral 
sense,  which  has  results  that  startle  those  who  have  been  successfully 
d?ceived.  Mental  change  is  for  the  most  part  absent  in  the  cases  due 
to  cold  (save  in  some  in  which  there  is  an  intense  blood-change) ,  and 
in  those  produced  by  metallic  poisoning ;  but  it  occurs  frequently  in 
toxaemic  cases,  generally  in  the  form  of  simple  delirium,  and  is  appa- 
rently due  to  a  simultaneous  action  of  the  morbid  agent  on  the  cells  of 
the  brain.  A  persistent  morbid  blood-state  may  cause  endocarditis 
and  other  consequences. 

Phthisis  is  also  a  very  frequent  complication ;  its  double  relation  to 
the  neuritis  has  been  ah-eady  mentioned.  The  form  of  lung  disease 
met  with  in  neuritis  presents  no  special  peculiarities,  except  that  low 
pneumonic  changes  play  a  considerable  part  in  the  morbid  process. 
Acute  pneumonia  is  also  common,  and  in  the  cases  in  which  it  does 
occur  almost  always  fatal. 

PATHOLoaicAL  Anatomt. — The  changes  in  the  nerves  correspond  to 
those  already  described  in  the  account  of  neuritis,  with  the  exception 
that  the  chief  changes  of  isolated  neuritis  are  in  the  connective  tissue, 
and  especially  in  the  sheath ;  and  those  in  the  nerve- fibres  are  subor- 
dinate. In  bilateral  neuritis  the  connective  tissue  presents  very  trifling 
changes.  Some  variations  are  met  with  in  this  respect ;  when  the  dis- 
tribution of  the  affection  is  not  strictly  symmetrical,  the  sheath  and 
interstitial  tissue  suffer  in  greater  degree  ;  in  proportion  to  the  acute- 
ness  and  symmetry  of  the  symptoms,  the  changes  are  restricted  to  the 
nerve-elements,  and  the  connective  tissue  is  unchanged,  but  in  some 
cases  both  the  connective  tissue  and  nerve-fibres  are  involved,  as  in 
Fig.  59.  This  is  also  the  condition  when  there  is  acute  inflamma- 
tion spreading  from  the  one  constituent  to  the  other,  as  in  the  case 
of  fatal  septicaemic  neuritis  recorded  by  Roth  (see  p.  148).  In  the  vast 
majority  the  nerve-elements  are  affected  chiefly  or  even  exclusively; 
the  changes  are  essentially  "parenchymatous."  It  is  remarkable, 
indeed,  how  slight  is  the  affection  of  the  connective  tissue  and  sheath, 
e^en  in  nerve-trunks  that  have  been  extremely  tender.  Hence  it  is 
probable  that  this  tenderness  is  often  due  to  changes  in  the  nerves  of 
the  sheath,  the  "  nervi  nervorum,"  similar  to  that  which  the  pn  per 
conducting  fibres  of  the  nerve  undergo,  rather  than  to  an  inflammation 
of  the  connective  tissue  itself. 

Naked-eye  changes  are  present  only  when  the  connective  tissue  and 
sheath  are  iaflamed  in  considerable  degree.  In  recent  and  acute  cases 
of  this  character  the  nerves  may  be  found  reddened,  swollen,  and 
sometimes  small  haemorrhages  are  visible.  In  older  cases  they  may  or 
may  not  be  swollen,  but  are  usually  softened,  and  even  pulpy.     When 


PATHOLOGICAL    ANATOMY.  1G5 

the  changes  are  confined  to  the  nerve-fibres,  diminished  consistence 
may  be  the  only  alteration  that  can  be  recognised  on  simple  examina- 
tion ;  and  often  this  is  absent,  and  the  nerves  appear  normal  on  external 
examination,  even  when  their  fibres  are  extensively  disintegrated. 
Sometimes  under  a  low  magnifying  power  a  section  of  the  nerve  has  a 
mottled  aspect,  due  to  the  irregular  destruction  of  the  fibres. 

When  the  connective  tissue  is  involved  the  microscope  shows  the 
sheath  to  be  infiltrated  with  lymphoid  cells,  and  in  old  cases  many 
spindle-cells  and  fibres  increase  the  bulk.  The  same  changes  may  be 
traced  in  the  septa  between  the  fasciculi,  and  in  the  secondary  sheaths 
that  surround  the  latter.  The  walls  of  the  vessels  are  also  thickened 
(Fig.  59,  a).  When  mere  acuteness  of  parenchymatous  neuritis  causes 
the  connective  tissue  to  be  inflamed,  the  interstitial  tissue  and  inner 
part  of  the  sheath  are  chiefly  affected,  and  an  amorphous  "  exudation  " 
may  be  found  in  these  situations.  But  the  fibres  themselves  always 
suffer  in  conspicuous  degree;  and  in  the  majority,  as  in  the  case  from 


V 


Pia.  59-— Multiple  alcoholic  neuritis;  sections  stained  with  carminp  and  cleared. 
A.  Transverse  section  of  part  of  sciatic  nerve;  low  power.  B.  Part  of  a  fas- 
ciculus of  same,  more  hig-hly  magnified.  0.  Part  of  a  less  affected  fasciculus 
from  a  musculo-spiral  nerve. 

which  Eigs.  69  and  60  are  drawn,  they  suffer  chiefly,  the  interstitial 
tissue  presenting  comparatively  little  change.  In  this  case  the  changes 
were  not  purely  toxic ;  there  was  a  complex  state,  combining  inflamma- 
tion of  the  fibrous  elements  of  the  nerves  with  the  purely  parenchyma- 
tous change.  The  patient  was  a  woman  aged  thirty-three,  alcoholic ; 
the  course  of  the  disease  was  subacute ;  the  symptoms  reached  a 
considerable  degree  in  a  few  weeks,  and  the  patient  died,  chiefly  from 
coincident  liver  disease,  five  months  after  the  onset.  The  symptoms 
were  characteristic,  and  continued  up  to  the  time  of  death.  The 
fibres  present  the  alterations  met  with  in  all  acute  forms  of  neuritis, 


166 


MULTIPLE    NEURITIS. 


essentially  tlie  same  as  tliat  wHcIl  occurs  after  an  injury  to  a  nerve, 
represented  in  Figs.  33  and  34  (p.  66),  and,  associated  with  inflam- 
mation of  tlie  sheath,  in  Fig.  44  (p.  83).  Examination  in  the  recent 
state  reveals  abundant  products  of  the  degeneration  of  the  fibres, 
granule  corpuscles,  &c.  There  is  a  slight  increase  in  the  connective 
tissue  between  the  fasciculi ;  the  area  of  these  is  occupied  by  ti-acts  of 
interstitial  tissue,  which  under  a  higher  magnifying  power  (b)  have  a 
branching  form,  enclosing  spaces  which  have  been  occupied  by  nerve- 
fibres,  and  in  some  of  which  healthy  fibres  still  remain  (c).  The  space 
between  this  tissue  is  occupied  by  the  products  of  degeneration  of  the 
fibres,  but  these  are  rendered  invisible  by  the  clearing  process,  and  only 
faint  indications  of  their  outline  can  be  seen  (b).  In  sections  stained 
with  osmic  acid  and  mounted  in  glycerine  (Fig.  60)  these  products  of 


Fia.  60. — Sections  from  the  same  sciatic  nerve,  stained  with  osmic  acid  and  mounted 
in  glycerine.  A.  Fasciculus  under  low  power.  B.  Part  of  a  fasciculus  more 
highly  magnified.  0.  Longitudinal  section  of  nerve-fibres.  (Prom  sections 
prepared  by  Dr.  Beevor.) 

degeneration,  stained  dark,  are  conspicuous.  Under  a  low  magnifying 
power  (a)  a  fasciculus  is  seen  to  be  studded  with  black  granular  spots, 
elongated  where  the  fibres  are  divided  obliquely ;  the  increase  in  the 
tissue  between  these  degenerated  fibres  is  distinct;  aggregations  of 
myelin  are  seen  in  the  connective  tissue  between  the  fasciculi.  Under 
a  higher  power  (b)  the  dark  spots  are  seen  to  be  the  sections  of 
degenerated  nerve-fibres,  in  some  places  two  or  more  being  blended 
into  a  larger  mass.  In  a  longitudinal  section  (c)  the  process  of 
degeneration  of  the  fibres  is  more  distinctly  seen,  and  from  it  the 
appearance  of  the  transverse  section  can  be  better  understood.  The 
white  substance  is  broken  up  and  enlarges  the  fibres  at  some  places, 
while  at  others  it  has  been  removed,  and  the  sheath  is  left  empty. 
Eound  masses  of  myelin  lie  (on  the  left)  outside  the  fibres.  The 
nuclei  of  the  fibres  are  enlarged ;  one,  of  considerable  size,  is  seen  in 
the  centre  of  the  figure.  Connective-tissue  fibres  and  cells  lie  between 
the  nerve-elements,  and  near  the  sheath  are  round  and  spindle  cells 
containing  myelin  granules  and  globules  which  they  have  taken 
up  (b). 

The  distribution  of  the  changes  in  the  nerves  varies  much  ia 
diferent  cases.     The  signs  of  inflammation  of  the  sheath  may  exist 


PATHOLOGICAL  ANATOMY.  167 

only  in  the  larger  and  medium  sized  nerves  of  tlie  limbs,  and  in  the 
smaller  nerves  the  changes  may  be  confined  to  the  nerve-fibres.  These 
may  be  traced  down  into  the  intra-muscular  nerves  and  their  endings. 
On  the  larger  nerves  the  signs  of  inflammation  cf  the  sheath  are  most 
intense  at  certain  spots,  especially  (as  in  simple  neuritis)  where  a 
nerve  turns  round  a  bone,  or  passes  through  a  fascia,  or  divides, 
or  in  the  neighbourhood  of  joints,  their  situation  being  probably 
determined  by  mechanical  influences.  As  a  very  rare  complication, 
changes  in  visceral  nerves  have  been  observed.  Severe  cohc  after 
influenza  was  found  to  be  attended  by  signs  of  inflammation  in  the 
nerves  and  gangha  in  the  abdomen.* 

In  all  cases  in  which  the  affection  is  chiefly  of  the  nerve-fibres  them- 
selves the  changes  are  usually  intense  in  the  peripheral  parts  of  the 
nerves,  and  as  the  nerves  are  examined  farther  from  the  periphery  the 
alterations  become  progressively  sHghter,  and  the  proportion  of  normal 
fibres  larger,  until  the  morbid  appearance  ceases.  The  centripetal 
extent  of  the  disease — that  is,  the  degree  in  which  the  larger  trunks 
are  affected — varies  according  to  the  duration  and  severity  of  the  case. 
If  the  nerve-fibres  could  be  isolated  and  seen  like  the  branches  and 
twigs  of  a  tree,  as  they  separate  in  the  peripheral  ramifications,  we 
should  see  the  endings  and  twig-like  branches  withered,  and  as  the 
branchlets  become  larger  and  larger  by  junction  they  would  assume 
a  more  and  more  normal  aspect  until  the  larger  branches  appear 
perfectly  natural.  The  size  of  branch  to  which  the  change  extended 
would  vary  according  to  the  severity  of  the  case.  In  some  instances 
even  those  of  largest  size  would  present  some  alteration,  while  in  others 
the  small  twigs  would  be  withered  in  a  considerable  part  of  the  tree, 
although  the  change  did  not  extend  beyond  them.  In  a  tree  the  leaves 
would  drop  off  equally  whether  the  twigs  only  were  diseased  or  the 
branches  also;  and  so  the  muscles  suffer  equally  whether  the  nerve 
degeneration  is  limited  to  the  periphery  or  extends  to  the  nerve-trunks. 
In  the  case  from  which  the  illustrations  are  taken  even  the  sciatic 
nerve  presented  few  healthy  fibres ;  but  here,  as  usual,  the  anterior 
roots  were  healthy.  This  limitation  to  the  peripheral  extremities  of 
the  nerves  is  met  with  in  slight  cases  of  considerable  duration,  but 
also  in  some  severe  cases  of  very  brief  duration.  For  instance,  in  a  case 
accompanied  by  pneumonia,  and  fatal  in  seven  days,  the  only  change 
found  was  in  the  fibres  themselves  within  the  muscles  ;  it  was  a  motor 
parenchymatous  neuritis.  The  character  of  the  changes  is  also  influenced 
by  that  of  the  nerve-fibres  involved.  If  the  affection  is  partial,  as  in 
the  ataxic  or  the  purely  motor  forms,  the  mixed  nerves  contain  many 
healthy  fibres  mingled  with  those  that  have  undergone  atrophy. 
When  the  symptoms  are  both  motor  and  sensory  almost  all  the  nerve- 
fibres  are  found  to  have  suffered. 

The  nerves  of  the  limbs  alone  present  changes  in  the  majority  of 
cases,  but  in  severe  and  acute  cases  almost  any  nerves  may  suffer,  and 
•  Ferguson,  'Alienist  and  Neurol.,'  1890. 


168  MULTIPLE    NEURITIS. 

this  in  wide  extent.  Tlie  corresponding  nerves  on  tlie  two  sides  aro 
almost  always  affected.  'Ihe  musculo-spiral  nerve  in  the  arm  and  the 
anterior  tibial  nerve  in  the  leg  usually  suffer  first  and  in  greatest 
degree,  but  often  all  the  terminal  nerves  of  the  limbs  are  involved  ia 
some  measure;  in  the  lower  leg  the  anterior  tibial  almost  always 
presents  more  change  than  the  other  nerves  of  the  same  level,  and  in 
the  thigh  the  sciatic  more  than  the  anterior  crural.  In  the  arm  the 
median  and  musculo-spiral  and  ulnar  all  suffer,  but  the  alteration  in 
these  nerves  in  the  upper  arm  may  be  slight  when  it  is  considerable  in 
their  branches  in  the  forearm.  The  changes  occasionally  met  with  iu 
other  nerves  correspond  to  their  implication,  as  described  in  the  section 
on  symptoms.  Various  irregularities  in  distribution  are  met  with  in 
cases  dependent  on  special  features  of  the  causal  toxine,  the  varieties 
of  which  are  extremely  numerous,  as  we  are  only  now  beginning  to 
perceive. 

Central  Changes  are  sometimes  met  with.  There  may  be  acute  or 
chronic  inflammation,  synchronous  effects  of  the  cause  of  the  neuritis. 
An  example  of  such  myelitis  is  figured  in  the  chapter  on  chronic 
myelitis  at  a  later  page.  More  important  and  at  present  more  obscure 
are  alterations  in  the  central  elements  on  which  the  nutrition  of  the 
neuron  depends,  of  the  same  parenchymatous  nature  as  the  peripheral 
changes.     Capillary  haemorrhages  have  also  been  found.* 

The  Muscles  present  changes  of  the  same  character  as  those  which 
result  from  ordinary  neuritis  (see  p.  70).  They  are  paler  than  normal 
and  smaller  in  bulk.  The  fibres  are  reduced  in  size,  and  pale ;  the 
transverse  striation  may  be  preserved,  or  they  may  be  granular; 
normal  fibres  may  be  found  side  by  side  with  those  that  are  degene- 
rated. The  nuclei  of  their  sheaths  and  of  the  interstitial  tissue  may  be 
increased  in  number,  and  often  are  arranged  in  groups ;  and  sometimes 
a  quantity  of  such  nuclei  and  leucocyte- like  cells  may  separate  widely 
the  fibres,  and  granular  and  pigment  masses  may  accumulate  between 
them.  In  very  acute  cases  the  changes  in  the  muscles  may  be  great, 
and  alike  parenchymatous  and  interstitial ;  the  fibres  presenting  loss 
of  their  striation  with  fatty  and  granular  degeneration,  while  there 
may  be  a  great  increase  in  the  amount  of  the  interstitial  tissue,  espe- 
cially of  its  nuolear  elements—:-"  acute  interstitial  myositis."t  This 
change  is  described  at  a  later  page  (p.  185). 

In  many  cases  there  has  been  merely  a  general  increase  in  the  con- 
nective tissue  throughout  the  cord,  which  has  been  in  some  instances 

*  Pal, '  Neur.  Cent.,'  1891.  He  also  found  degeneratiou  iu  the  lateral  and  posterior 
columns,  and  similar  alterations  have  been  since  described  by  others. 

Ballet,  *  Prog.  Med.,'  1896,  Nos.  18 — 26.  A  controversy  has  arisen  such  as  to 
cause  some  French  observers  to  be  termed  "  centriiliste,"  and  others  "  peripheraliste," 
the  one  holding  that  the  cell  is  the  first  to  be  affected,  and  that  the  peripheral  ))art 
suffers  secondarily ;  while  the  others  maintain  that  the  normal  aspect  of  the  nerve- 
roots  is  incompatible  with  this  view.  Ballet  discusses  the  question,  but  without 
deciding  it. 

t  See  Senator,  '  Zeitschr,  f.  kl.  Med.,'  1889. 


PATHOLOGY.  169 

greater  in  one  region  than  elsewhere,  and  especially  marked  in  the 
posterior  columns.  Such  alterations  are  most  marked  in  chronic  cases 
in  which  the  symptoms  have  continued  for  months,  and  especially  in 
those  due  to  chronic  alcoholism,  or  in  which  life  has  been  prolonged  in 
a  state  of  disability. 

Other  organs  are  often  found  diseased,  and  in  alcoholism  the  lesions 
common  in  that  disease  are  generally  found  in  some  degree,  especially 
alterations  in  the  liver  and  kidneys.  The  former  may  be  large  and 
either  cirrhosed  or  fatty ;  the  kidneys  are  generally  large  and  opaque, 
sometimes  with  fatty  striae  in  the  cortex.  Pneumonia  is  frequent 
both  in  these  and  in  all  cases  due  to  causes  that  induce  an  intense 
change  in  the  blood.  In  these  there  may  also  be  enlargement  of  the 
spleen,  such  as  is  met  with  in  typhoid  fever  and  septicaemia.  Phthisical 
changes  in  the  lungs  are  also  common  in  chronic  cases,  both  in  those 
that  are  due  to  alcohol  and  in  others.  No  special  peculiarity  has  been 
observed  in  the  pulmonary  lesion,  but  it  is  very  desii'able  that  more 
attention  should  be  paid  to  the  state  of  the  nerves  of  the  langs 
entering  the  diseased  parts.  When  not  otherwise  diseased,  the  lungs 
are  generally  found  to  be  the  seat  of  terminal  congestion  and  oedema. 
The  walls  of  the  heart  may  be  degenerated,  and  alterations  may  some- 
times be  found  in  its  nerves. 

Pathology. — An  outline  of  the  chief  facts  in  the  pathology  of  mul- 
tiple neuritis  has  been  given  in  the  introductory  section,  and  not  much 
remains  to  be  added.  The  relation  of  the  motor  symptoms  to  the  lesion 
of  the  nerves  has  also  been  described  in  the  general  account  of  diseases 
of  the  nerves,  and  in  the  description  of  isolated  neuiitis  ;  all  that  has 
been  said  of  the  single  form  is  true  also  of  that  which  is  multiple. 
The  wasting  that  accompanies  the  loss  of  power,  and  the  change  in 
electrical  irritability,  are  the  same  in  each.  In  each  it  is  true,  more- 
over, that  the  position  of  the  lesion  in  the  course  of  the  nerve-fibre 
makes  no  difference  to  the  symptoms ;  these  are  the  same  whether  the 
disease  is  in  the  trunk  of  the  nerve,  the  peripheral  fibres,  or  the  actual 
nerve-endings.  The  relation  of  the  ataxy  in  the  pseudo-tabetic  form 
to  a  neuritis  of  the  afferent  muscular  nerves  is  proved  partly  by 
exclusion,  partly  by  analogy,  since  in  true  tabes  the  lesion  may  be 
confined  to  these;  the  spinal  cord  may  be  free,  and  only  the  peri- 
pheral nerves  diseased.  The  evidence  for  this  will  be  described  in  the 
general  account  of  the  functions  and  symptoms  of  diseases  of  the 
spinal  cord,  and  in  the  chapter  on  locomotor  ataxy — a  disease  which 
sometimes  not  only  resembks,  but  is  almost  identical  with,  the  pseudo- 
tabetic  form  of  neuritis.  The  pains  and  hypersesthesia  are  explained 
by  the  fact  that  the  disease  of  the  nerves  is  one  in  which  the  nerve- 
fi.bres  themselves  suffer  primarily,  and  that  on  them  the  cause  must 
have  a  primary  and  direct  action.*    An  influence  which  leads  to  the 

*  In  the  first  edition  1  suggested  that  the  degeneration  of  some  of  the  fibres 
might  be  secondary  to  their  damage  by  the  inflammation  of  the  sheath  and  inter- 
•titial  tissue  at  some  higher  spot.     But   this   now  seems  very  unlikely;  it  is  far 


170  MULTIPLE    NEURITIS. 

molecular  destruction  of  the  nerve-fibres  must,  in  destroying  their 
structure,  disturb  gravely  their  function;  and  this  disturbance  of 
structure  and  of  function  proceeds  in  the  parts  still  connected  with 
the  nerve-centres,  so  that  the  irritative  influence  of  the  molecular 
changes  can  act  on  the  centres  by  the  normal  upper  parts  of  the 
nerves  throughout  the  whole  course  of  the  malady.  It  induces  in  the 
centres,  as  all  prolonged  pain  does,  an  undue  readiness  of  action,  a 
state  of  over-excitability;  and  so  we  can  understand  the  persistence 
of  the  pains  and  hypersesthesia,  and  the  fact  that  these  continue 
until  the  process  of  restoration  has  become  definitely  established. 
The  same  considerations  apply  to  the  tenderness  of  the  muscles,  since 
the  afferent  muscular  nerves  correspond  in  the  direction  of  their 
function  to  the  sensory  nerves  of  the  skin.  In  both  cases  mechanical 
influences  constitute  the  chief  mode  of  their  normal  stimulation,  and 
hence  they  may  well  be  morbidly  sensitive  to  such  influences.  The 
peripheral  distribution  of  the  changes  indicates,  moreover,  that  pro- 
bably the  first  to  suffer  are  those  in  which  functional  susceptibility 
is  most  highly  developed — the  actual  endings  of  the  nerves,  and  the 
structures  which  are  especially  adapted  for  the  reception  of  stimuli 
and  the  production  of  nerve-force. 

We  have  seen  that  two  facts  give  us  the  key  to  the  pathology  of 
the  disease  so  far  as  this  is  yet  understood.  One  is  its  symmetry ; 
the  other  is  the  fact  that  it  is  commonly  a  disease  of  nerve- fibres — 
that  these,  and  not  the  connective  tissue,  are  the  parts  primarily  and 
specially  diseased.  In  all  structures,  in  proportion  to  the  intensity 
and  acuteness  of  the  parenchymatous  affection,  the  interstitial  tissue 
participates  in  the  changes,  and  escapes  in  proportion  to  its  slowntss 
and  slightness.  The  extent  to  which  this  is  true  of  multiple  neuritis 
has  been  already  pointed  out.  Further,  in  its  relation  to  a  morbid 
blood-state  as  its  sole  cau^e,  we  have  the  key  to  the  symmetry  of 
the  affection  as  well  as  to  its  parenchymatous  nature,  and  on  this 
point  there  remains  little  to  add  to  what  has  been  already  said. 
Such  a  limitation  to  the  nerve-fibres  as  is  seen  in  multiple  neuritis 
is  unknown  in  the  isolated  form,  in  which,  as  in  gouty  or  syphilitic 
neuritis,  the  affection  is  chiefly  one  of  the  sheath  and  interstitial 
connective  tissue;  and  the  fibres  suffer  only  secondarily  in  chronic 
cases,  although,  in  accordance  with  the  law  abeady  stated,  they  are 
involved  in  acute  cases  in  proportion  to  the  intensity  of  the  process. 

It  is  not  surprising  that  the  symptoms  of  polyneuritis  should 
bear  so  close  a  resemblance  to  those  of  some  affections  of  the  spinal 
cord,  since  we  must  regard  the  peripheral  nerves,  at  least  the  motor 
fibres,  as  essentially  outlying  parts  of  the  cord,  and  it  will  be  seen 
that  one  central  disease,  primary  lateral  sclerosis,  presents  many 
points  of  correspondence  with  the  chronic  form  of  polyneuritis,  being 

more  probable  tbat  all  the  fibres  sufPer  in  the  same  way,  and  the  more  thoroughly 
cases  are  studied  the  more  clearly  does  it  appear  that  all  symmetrical  neuritis  is 
peripheral  and  parenchymatous. 


PATHOLOGY.  171 

an  affection  of  the  fibres  proceeding  from  tlie  motor  cells  of  tlie  brain 
analogous  to  that  of  the  fibres  proceeding  frora  the  motor  cells  of  the 
cord.  We  can  understand  the  peripheral  distribution  of  the  disease 
by  the  fact  already  pointed  out — that  the  nerve-fibres  are  really  the 
prolonged  processes  of  the  nerve-cells,  sharing  the  life  of  the  cell,  and 
depending  on  it  for  their  vitaUty.  The  influence  on  which  their  life 
depends  must  lessen  with  the  distance  from  its  source,  and  hence  their 
vital  power  of  resisting  morbid  agencies  is  least  at  the  periphery. 
The  conditions  of  life  of  the  sensory  fibres,  it  need  hardly  be  pointed 
out,  differ  little  from  those  of  the  motor,  since  the  ganglia  containing 
their  cells  are  near  the  cord,  and  almost  as  far  from  the  periphery  as 
are  the  motor  nerve -cells.  This  fact  also  has  a  relation  to  the  paren- 
chymatous nature  of  the  disease,  since  we  can  find  no  reason  why  the 
connective  tissue  of  the  nerves  should  suffer  most  at  the  extreme 
periphery.  On  the  contrary,  the  inflammations  that  begin  in  the 
sheath  are  generally  situated  in  the  course  of  the  nerves,  at  some  spot 
exposed  to  the  influence  of  an  exciting  cause  such  as  pressure  or  motion. 
The  same  fact  (that  the  cause  is  a  blood-state)  affords  also  an  explana- 
tion of  many  facts  of  distribution.  In  addition  to  the  points  already 
mentioned  it  is  only  necessary  to  point  out  how  common  are  instances 
of  selective  action  of  toxio  agents  upon  various  parts  of  the  nervous 
system  ;  and  analogy  prevents  us  from  feeling  any  difiiculty  in  at  least 
accepting  the  fact  that  a  blood-state  should  act  upon  one  set  of  nerve- 
fibres  rather  than  upon  another,  or  on  the  fibres  for  the  extensors,  or 
even  first  and  chiefly  on  only  some  of  the  nerve-fibres  of  a  certain  set 
of  muscles.  We  do  not  yet  know  what  are  the  influences  that  deter- 
mine such  susceptibility,  but  it  is  probable  that  a  very  sHght  difference 
in  nature  is  sufiicient  to  determine  a  very  great  difference  in  the  result. 
The  fact  of  symmetry  excludes,  as  we  have  seen,  any  other  causal 
influences  than  those  which  act  through  the  blood,  or  consist  in  a  vital 
tendency,  and  act  on  all  structures  with  similar  vital  conditions.  We 
know  nothing  as  yet  of  the  precise  character  of  the  morbid  influence 
which  causes  the  malady,  beyond  the  facts  mentioned  in  the  intro- 
ductory section.  'Ihe  greatest  difficulty  is  presented  by  the  fact  that  a 
poison  generated  through  exposure  to  cold,  or  possibly  by  a  growth  of 
organisms  in  the  body,  should  have  an  influence  similar  to  that  which 
is  exerted  by  metals  or  by  alcohol.  At  the  same  time  it  is  instructive 
to  note  that  the  most  recent  researches  in  bacteriology  point  to  the  pro- 
duction of  chemical  substances  by  the  disease-causing  organisms  in  the 
com-se  of  their  growth,  and  suggest  that  these  chemical  products, 
rather  than  the  organisms  themselves,  act  upon  the  nerves.*  Alcohol, 
it  may  be  further  noted,  is  itself  the  result  of  the  growth  of  organisms 
almost  as  low  in  the  scale  as  those  which  are  the  causes  of  disease ;  the 
process  of  the  growth  of  bacteria  in  the  body  may  be  not  unlike  a 
process  of  fermentation,  and  the  results  of  the  one  not  unlike  those  or 
the  other.  The  analogy  is  the  more  pertinent,  since  multiple  neuritis 
*  A  theory  apparenily  tirst  suggested  by  Kosenheim, '  Arch.  f.  Psych.,'  xviii,  3. 


172  MULTIPLE   NEURITIS. 

is  not  known  to  be  excited  by  any  other  organic  poison  than  that 
which  is  so  produced. 

The  forms  of  polyneuritis  ascribed  to  anaemia  and  cachexia,  &c., 
may  be  a  simple  failure  of  nutrition  in  those  structures  of  the  nervous 
system  that  are  farthest  removed  from  the  centres ;  but  the  possibility 
must  not  be  forgotten  that  imperfect  tissue  changes  may  generate  a 
toxic  agent  capable  of  acting  on  these  nerves,  analogous  to  that 
assumed  to  be  effective  in  diabetes.* 

The  changes  in  nutrition  are  sufficiently  explained  by  the  alterations 
found  in  the  nerves,  in  accordance  with  the  general  facts  of  trophic 
disturbance  stated  at  pp.  27  and  65.  That  in  the  muscles  corresponds 
in  distribution  to  the  changes  in  the  motor  nerves,  that  in  the  skin  and 
joints  to  those  in  the  sensory  nerves.  All  trophic  changes  which 
resiilt  from  ordinary  nerve-lesions  must  be  ascribed  to  the  propagation 
downwards  of  the  influence  of  the  defective  and  abnormal  nutrition ;  and 
hence  it  necessarily  follows  that  such  abnormal  nutrition,  beginning  in 
the  nerve-endings,  should  have  a  similar  influence,  since  it  is  through 
those  nerve-endings  that  the  effects  of  higher  lesions  are  transferred 
to  the  tissues.  The  law  already  stated  holds  good  here  also, — that 
changes  in  nutrition  are  in  proportion  to  the  intensity  and  acuteness  of 
the  disturbance  in  the  nerves.  All  that  has  been  said  regarding  such 
changes  in  the  sldn,  in  diseases  of  isolated  nerves,  is  true  also  of  the 
multiple  form,  and  even  more  clearly  in  the  case  of  the  motor  nerves 
and  muscles.  The  more  acute  the  process  in  the  nerves,  the  more 
intense  is  the  change  in  the  muscular  tissue.  One  special  point,  indeed, 
arises  in  this  connection.  Although,  in  very  acute  isolated  neuritis, 
the  muscles  may  undergo  the  same  intense  and  destructive  changes 
seen  in  the  most  acute  forms  of  multiple  neuritis,  it  is  possible  that 
the  extreme  changes  in  the  latter  are  sometimes  a  result  and  evidence 
of  a  direct  action  of  the  blood-poison  on  the  muscular  tissue  itself,  t 
There  are  some  facts  of  both  physiology  and  pathology  which  suggest 
what  may  be  called  a  certain  degree  of  solidarity  of  relation  between 
the  substance  of  the  nerve-endings  and  that  of  the  muscular  fibres  on 
which  they  terminate.  This  may  lead  to  a  common  susceptibility  to 
suffer  from  the  same  morbid  influence,  so  far  less  in  the  muscle  that 
only  when  the  influence  is  very  intense  does  its  nutrition  suffer  directly 
to  a  considerable  extent. 

Diagnosis.— There  are  few  diseases  in  which  an  early  diagnosis  is 
of  greater  importance.  Prompt  treatment  would  save  many  patients 
from  months  of  suffering  and  disability,  since  the  removal  of  the  cause 

*  This  analogy  is  borne  out  by  the  occurrence  of  degenerative  changes  in  the 
epinal  cord,  occurring  in  association  with  profound  anaemia,  as  described  by  Licht- 
heim  and  others. 

t  This  suggestion  has  been  made  by  Soemerling  as  an  explanation  of  the  very 
intense  changes  found  in  the  muscles  in  one  acute  case.  In  beri-beri  the  change  in 
the  cardiac  muscle  is  not  infrequently  very  great,  and  quite  out  of  proportion  to 
that  found  in  its  nerves. 


DIAGNOSIS.  173 

ensures  with  certainty,  during  a  gradual  onset,  a  quick  removal  of  the 

aymptoms,  whereas  its  effect  on  the  developed  disease  is  manifested 
much  more  slowly.  These  remarlvs  apply  especially  to  the  alcoholic 
cases,  but  in  some  of  the  others  the  patient  may  be  saved  further 
exposure  to  the  cause  if  the  disease  is  promptly  recognised,  or  early 
treatment  of  the  blood-state  may  have  a  considerable  influence  in 
lessening  its  effects  upon  the  nerves  and  ameliorating  the  course  of  the 
affection. 

The  diagnosis  of  multiple  neuritis  is  becoming  far  less  liable  to  error. 
It  depends  on  the  motor  and  sensory  symptoms  above  described,  their 
correspondence  in  function  and  distribution  ia  the  opposite  limbs, 
their  pecTiliar  symmetrical  localisation  in  the  extremities  in  the  first 
instance,  and  on  the  tenderness  of  the  skin,  nerve-tronks,  and  muscles. 
Symmetrical  weakness  of  the  extensor  muscles  situated  in  the  forearm, 
and  of  the  corresponding  muscles  in  the  lower  leg,  or  of  either  of  these, 
is  the  leading  diagnostic  motor  symptom.  Bilateral  wrist- drop  or 
foot-drop  suggests  multiple  neuritis,  and  hyperaesthesia  or  deep  tender- 
ness gives  strength  to  the  opinion.  It  is,  however,  most  important  to 
remember  how  widely  the  symptoms  vary  both  ia  different  forms  and 
in  different  cases  of  the  same  form.  The  acute  rapidly  fatal  cases,, 
with  palsy  quickly  becoming  general,  are  of  a  type  quite  Tinlike  the 
chronic  cases  in  which  one  group  of  muscles  on  each  side  suffers  alone ; 
while  there  is  a  positive  contrast  between  the  latter  and  a  case  of 
"  pseudo-tabes,"  in  which  ataxy  and  pains  are  almost  the  only  sym- 
ptoms. In  chronic  cases  the  early  pains  are  often  mistaken  for  those 
of  rheumatism,  and  in  those  of  acute  course,  especially  when  due  to 
cold,  these  pains  suggest  the  onset  of  rheumatic  fever  rather  than  of 
any  affection  of  the  nerves.  The  seat  of  the  pains,  however,  is  not 
that  of  rheumatic  pain,  and  their  association  with  tingling  in  the 
extremities  should  excite  a  suspicion  that  they  are  of  nerve  origin  even 
before  muscular  weakness  renders  their  nature  clear.  It  must  be  again 
pointed  out  that  pain  near  a  joint,  produced  by  movement  of  the 
joint,  is  easily  mistaken  for  pain  in  the  joint,  even  when  it  depends 
entirely  on  tenderness  of  the  adjacent  nerves.  In  some  cases  the  pain 
is  regarded  as  neuralgic,  especially  when  it  is  seated  in  the  arms  and 
hands,  and  is  perceived  to  be  in  the  nerve-trunks  or  branches.  But 
the  general  elements  of  the  diagnosis  between  neuritis  and  neuralgia 
usually  suffice  for  the  distinction,  the  most  important  point  being  the 
persistence  of  tenderness  and  hypersesthesia  in  neuritis,  relative  excess 
of  the  spontaneous  pain,  and  also  the  development  of  symptoms  that 
indicate  damage  to  the  conducting  fibres.  The  bilateral  symmetry  of 
the  pain  should  also  help  to  prevent  error ;  true  neuralgia  is  essentially 
a  unilateral  disease.  A  gouty  diathesis  may  increase  the  difficulty  of 
the  diagnosis,  especially  in  cases  of  alcoholic  neuritis  ;  but  the  pains 
of  gout  are  unequivocally  articular  or  muscular. 

The  symptoms  resemble  most  closely  those  of  certain  diseases  of  the 
spinal  cord,  especially  acute  and  subacute  inflammation  of  the  grey 


174  MULTIPLE    NEURITIS. 

mattei* — polio-myelitis.  Until  recently,  indeed,  all  cases  of  multiple 
neuritis  were  looked  upon  as  of  that  nature.  It  is  chiefly  in  the  rheu- 
matic and  toxasmic  forms  that  the  diagnosis  is  really  difficult.  In  each 
disease  there  may  be  a  febrile  onset,  muscular  wasting  with  the  reac- 
tion of  degeneration,  initial  rheumatic  pains,  and  a  tendency  to  the 
spontaneous  recovery  of  the  least  affected  parts.  The  distinction  rests 
on  the  symmetrical  localisation  of  the  neuritic  palsy,  while  that  of 
polio-myelitis  is  characteristically  random  ia  distribution ;  on  the 
persistence  and  severity  of  the  neuritic  pains ;  on  the  tenderness  of 
the  inflamed  nerve-trunks  ;  and  on  the  changes  in  sensibility,  a  symptom 
never  present  in  polio-myelitis.  The  electrical  reactions  are  the  same 
in  both  affections.  It  has  been  said  the  early  extinction  of  voltaic 
iriitability  in  the  muscle  (see  p.  72)  is  met  with  only  in  disease  of 
the  nerves,  but  this  is  incorrect;  I  have  more  than  once  met  with  it 
in  central  affections.  But  we  have  seen  that  it  is  probable  that 
the  nerve-trunks  are  sometimes  inflamed  in  polio -myelitis,  and  suffer 
as  a  simultaneous  effect  of  the  cause  of  the  spinal  lesion.  In  this  con- 
dition, however,  the  spinal  symptoms  preponderate,  and  the  distri- 
bution of  the  neuritis  is  irregular.  In  all  cases  the  presence  of 
increased  myotatic  irritability,  or  of  the  spasm  in  the  legs  that  accom- 
panies considerable  excess  of  this,  may  be  taken  as  proof  of  disease  of 
the  cord;  all  active  muscular  spasm,  indeed,  has  this  significance. 
Thus  if  the  arms  are  paralysed,  even  though  the  paralysis  closely 
resembles  that  of  polyneuritis,  and  the  legs  are  weak,  with  excessive 
knee-jerk  and  foot- clonus,  the  disease  is  of  the  cord,  and  not  of  the 
nerves.  The  greatest  difficulty  is  presented  by  some  sensory  cases 
in  which  there  are  only  tingling  and  numbness  in  the  legs,  and 
also  by  the  problem  of  detecting  cord  disease  in  the  presence  of 
peripheral  neuritis.  The  latter  will  be  considered  further  on.  The 
former  is  generally  decided  by  the  presence  or  absence  of  the  knee- 
jerk,  and  by  the  fact  that  in  peripheral  neuritis  the  sensory  symptoms 
are  more  peculiar  and  symmetrical  than  in  cord  disease;  there  is 
tingling  or  anaesthesia,  for  instance,  in  the  palms  or  soles,  or  both, 
and  a  careful  examination  of  the  nerves  and  m.uscles  will  generally 
reveal  a  condition  that  is  characteristic. 

Pachymeningitis,  damaging  the  nerve-roots,  m.ay  cause  paralysis, 
wasting,  and  ansesthesia,  but  in  this  all  four  limbs  are  not  affected; 
the  legs  rarely  suffer ;  the  anaesthesia  invades  the  upper  parts  of  the 
limbs  or  the  trunk  as  much  or  more  than  the  distal  parts ;  there  is  no 
tenderness  of  nerve-trunks,  and  there  is  usually  distinct  evidence  of 
damage  to  the  spinal  cord  itself.  The  mysterious  disease,  "  acute  ascend- 
ing paralysis,"  may  resemble  the  most  rapid  form  of  multiple  neuritis, 
but  in  it  the  symptoms  ascend  the  trunk  from  the  legs  to  the  arms,  and 
do  not  begin  in  the  hands  and  feet  at  the  same  time  or  successively,  and 
involve  the  trunk  last,  as  does  the  usual  form  of  multiple  neuritis. 
Many  cases,  perhaps  indeed  most,  are  of  toxic  origin,  and  are  really 
neuritis.     Diphtheritic  paralysis  is  of  uncertain  nature,  but  there  is  no 


DIAGNOSIS.  175 

considerable  pain,  and  the  weakness  in  the  limhs  usuallj  succeeds 
paralysis  of  the  palate  and  ciliary  muscle,  never  met  with  in  other 
forms  of  polyneuritis.  The  distinction  from  other  diseases  (as,  for 
instance,  that  from  the  symmetrical  paralysis  of  the  extensors  which 
may  occur  at  the  onset  of  progressive  muscular  atrophy)  will  be  con- 
sidered when  these  diseases  are  described. 

The  distinction  of  polyneuritis  in  its  ataxic  form,  "pseudo-tabes," 
from  true  tabes  is  often  beset  with  great  difficulty.  This  is  indeed 
natural,  since  the  lesion  in  true  tabes  may  be  identical  with  that  in  the 
neuritic  affection ;  not  only  is  degeneration  of  the  peripheral  nerves  a 
common  lesion  in  tabes,  having  the  same  distribution  at  the  peripheral 
extremities  of  the  ne.ves  as  in  polyneuritis,  but  in  the  variety  termed 
"  neuro-tabes  "  the  lesion  consists  only  in  this  nerve  degeneration,  and 
the  spinal  cord  is  free  from  disease.  We  have,  then,  affections  iden- 
tical in  pathological  anatomy,  and  almost  identical  in  symptoms. 
Yet  we  must  class  the  neuro-tabes  with  the  spinal  form,  from  which 
polyneuritis,  as  here  described,  has  to  be  distinguished ;  it  is  necessary 
to  do  so  on  account  of  the  relations  and  etiology  of  the  two  maladies 
as  well  as  on  account  of  practical  considerations.  The  presence  of 
actual  paralysis,  of  distinct  weakness  of  the  extensors,  in  addition  to 
inco-ordination,  would  of  course  decide  the  question,  since  there  is  no 
actual  loss  of  power  in  tabes :  hence  a  distinction  in  gait  pointed  out 
by  Westphal  and  Charcot,  that  in  pseudo-tabes  the  feet  are  raised  too 
high  on  account  of  the  difficulty  of  getting  the  toes  off  the  ground 
(like  a  person  stepping  over  low  objects),  is  of  little  actual  value ;  it 
only  exists  when  there  is  distinct  loss  of  power.  But  it  is  in  the  cases 
of  neuritis  in  which  there  are  only  sensory  symptoms  that  |;he 
difficulty  especially  exists.  It  occurs,  moreover,  chiefly  when  the  ataxy 
is  moderate  in  degree ;  when  this  is  so  great  that  the  patient  is  not 
able  to  stand  alone,  in  spite  of  the  power  of  moving  all  joints  of  the 
legs  with  fair  force,  true  tabes  should  alone  be  thought  of.  The  cases 
of  tabes  in  which  there  is  moderate  inco-ordination  of  movement, 
distinct  uno.eadiness  on  walking,  whether  increased  by  closure  of 
the  eyes  or  not,  and  distinct  irregularity  and  uncertainty  in  the  move- 
ment of  the  feet  and  legs,  are  those  which  are  joerfectly  simulated  by 
the  pseudo-tabetic  form  of  polyneuritis.  Pains  are  common  to  the  two 
diseases,  and  neither  prolonged  acute  pain  nor  dull  rheumatoid  pains 
afford  the  means  of  distinction.  True  "lightning"  pains  are  seldom 
met  with  in  neuritis ;  they  are  therefore  strong  evidence  that  the  case 
is  one  of  tabes.  Extreme  hyperaesthesia  is  much  more  common  in 
neuritis  than  in  tabes,  but  is  of  little  service  in  the  diagnosis  because 
it  is  not  often  present  in  ataxic  polyneuritis,  but  the  muscular  tender- 
ness of  neuritis  is  of  considerable  diagnostic  value.  A  great  excess  of 
tJie  reflex  action  from  the  skin  is  occasionally  met  with  in  tabes,  the 
sensory  impressions  being  painless  ;  while  in  ataxic  neuritis  the  reflex 
action  is  seldom  excessive,  and  is  generally  diminished,  while  the 
sensory    impression,  is  commonly  painful  from  the   hyperaesthesia. 


176  MULTIPLE    NEUUITIS. 

Some  other  symptoms  are  also  of  value  as  indications  of  tahes  rather 
than  neuritis, — an  affection  of  the  sphincters ;  a  sense  of  constriction 
round  the  trunk  (which  may  be  felt  over  a  wide  area  as  a  band  rather 
than  as  a  cord)  ;  and  lastly  (but  among  the  first  in  importance),  the 
reflex  action  of  the  pupil  to  light  is  often  lost  —  seldom  if  ever  in  neuritis. 
Since  it  may  be  normal  in  tabes,  its  loss  is  of  more  significance  than 
its  presence,  but  the  cases  of  tabes  in  which  it  is  preserved  form  only  a 
small  minority,  so  that  its  preservation  is  often  at  least  corroborative 
evidence  that  the  case  is  one  of  polyneuritis.  Mental  change  is  of  little 
value  in  the  diagnosis  unless  it  is  in  the  form  of  definite  delirium  ; 
but  the  less  common  symptoms  of  tabes,  if  present,  often  afford  proof 
of  the  nature  of  the  malady,  although  generally  in  association  with 
other  symptoms  rather  than  by  themselves.  Optic  nerve  atrophy,  for 
instance,  is  almost  unknown  in  the  neuritic  "  pseudo-tabes,"  and  so 
also  are  the  various  "crises:"  care  must  betaken  not  to  confound 
the  vomitiu'^y  of  alcoholic  gastric  disturbance  with  the  gastric  crises  of 
tabes.  The  trophic  changes  differ  in  the  two  :  the  tabetic  enlargement 
of  the  bones  and  disease  of  the  joints  are  unknown  in  neuritis  ;  and 
so,  in  the  former  affection,  are  the  simple  arthritic  adhesions  and  the 
"  glossy  skin "  of  the  latter.  If  all  the  symptoms  are  considered 
separately  for  their  individual  value,  and  conjointly  for  their  associated 
significance,  it  is  not  often  that  the  observer  will  remain  in  doubt. 

One  other  special  diagnosis  should  be  mentioned,  that  for  hysterical 
palsy.  This  occurs  chiefly  in  women  when  the  symptoms  are  motor 
and  are  in  the  legs.  When  in  the  arms,  the  characteristic  situation 
of  the  palsy  usually  attracts  attention  and  prevents  error.  A  defect 
in  the  power  of  extension  of  the  wrist  has  probably  never  been  met 
with  as  a  consequence  of  hysteria.  Slight  ataxy  may,  however,  be 
purely  functional  in  nature,  but  the  knee-jerk  is  not  lost,  and  if  there 
is  anaesthesia  it  is  much  more  extensive  in  the  functional  affection. 

It  is  of  great  importance  to  know  whether  the  spinal  cord  is  suffer- 
in  o-  as  well  as  the  nerves.  The  following  symptoms  are  those  that  are 
the  most  common  and  important  evidence  of  this  complication  : — (1) 
Any  impairment  of  the  functions  of  the  bladder  or  rectum — inconti- 
nence of  faeces,  retention  or  incontinence  of  urine.  It  is  not,  indeed, 
improbable  that  their  impairment  may  form  part  of  the  most  intense 
'  form  of  polyneuritis,  but  it  is  not  part  of  the  ordinary  phenomena  of 
the  disease ;  hence,  in  such  cases,  these  symptoms  suggest  that  the 
disease  involves  the  cord  as  well  as  the  nerves.  It  is  important  to  re- 
member that  when  there  is  mental  change,  evacuations  into  the  bed 
are  frequently  without  significance  as  to  the  state  of  the  sphincters, 
and  are  due  solely  to  the  inertia  of  the  mind.  This  is  a  fertile  source 
of  error.  (2)  A  distinct  sense  of  constriction,  "  girdle-pain,"  referred 
to  any  part  of  the  trunk,  probably  always  constitutes  proof  of  damage 
to  the  cord.  Of  the  same  significance  is  sharp  pain  radiating  around 
the  trunk  at  a  definite  level  and  not  elsewhere.  (3)  An  important 
distinction  in  alcohohc  cases  is  an  unusual  distribution  of  the  affeo- 


DIAGNOSIS.  177 

tion,  e.  g.  complete  paralysis  of  all  parts  of  tlie  legs,  or  weakness  of 
the  muscles  above  the  knee,  and  not  of  those  below.  Impairment  of 
sensation  up  to  a  certain  level  on  the  legs  or  trunk  has  the  same  sig- 
nificance ;  disease  of  the  nerves  does  not  involve  those  of  sensation  up 
to  a  definite  level.  Bedsores  also  increase  the  probability  that  the  cord 
is  affected. 

If  a  case  is  recognised  as  one  of  multiple  neuritis  there  remains  the 
question,  what  is  its  cause  ?  In  many  cases  this  question  does  not 
arise,  because  the  cause  is  obtrusive,  and  has  been  recognised  as  one 
of  the  elements  of  the  diagnosis ;  but  in  other  cases  the  cause  is  not 
clear,  the  diagnosis  has  been  made  independently  of  it,  and  it  remains 
to  be  discovered.  For  the  most  part  it  is  only  necessary  to  know  the 
various  causes  in  order  to  determine  which  is  effective  in  the  patient 
under  observation,  because  one  or  another  of  them  is  to  be  discovered 
without  difficulty,  and  the  rest  are  absent.  It  may  be  useful,  however, 
to  remember  that,  as  a  rule,  in  chronic  metallic  poisoning  the  arms 
suffer  before  the  legs,  and  that  in  lead  poisoning  the  acute  paralysis 
is  limited  to  the  arms ;  in  arsenical  poisoning  the  affection  of  the  arms 
is  soon  followed  by  that  of  the  legs,  while  in  alcoholic  cases  the  legs 
generally  suffer  first.  In  a  case  in  which  arms  and  legs  are  affected 
simultaneously  metallic  poisoning  woxdd  be  a  very  unlikely  cause.  An 
initial  affection  of  the  proximal  parts  of  the  limbs  at  the  same  time  as 
the  distal  parts  suggests  some  other  cause  than  alcohol,  especially  cold 
or  toxaemia.  Early  affection  of  the  muscles  of  the  trunk  or  of  the 
bulbar  nerves  has  the  same  significance,  and  so  also  has  severe  con- 
stitutional disturbance  at  the  outset.  It  must  be  also  remembered, 
however,  that  alcoholism  often  co-operates  with  other  causes,  especially 
with  cold  ;  and  it  must  also  be  remembered  that  the  inquiry  needs  to 
be  exhaustive  before  the  influence  of  alcohol  can  be  excluded.  This  is 
especially  true  in  the  case  of  females,  who  often  obstinately  deny  that 
which  would  bring  shame  upon  them.  Exposure  to  cold  has  generally 
been  severe  ;  and  if  it  has  not,  the  co-operation  of  alcohol  is  extremely 
probable.  Neuralgic  pains  of  irregular  distribution  in  addition  to  the 
symptoms  of  polyneuritis  should  suggest  diabetes  as  a  cause,  but  the 
examination  of  the  urine  for  sugar  should  never  be  omitted.  "When 
polyneuritis  is  due  to  septicaemia  this  cause  may  be  suggested  by  the 
fact  that  the  patient  is  under  treatment  for  some  surgical  ailment ; 
while  in  all  cases  of  obscure  origin  inquiry  should  be  made  for  any 
acute  specific  disease  to  which  the  neuritis  is  known  to  be  secondary, 
and  the  fact  should  be  remembered  that  a  considerable  interval  may 
separate  the  two.  Some  form  of  toxaemia  is  always  suggested  by  the 
fact  that  the  onset  of  the  neuritis  is  associated  with  such  pyrexia  and 
constitutional  disturbance  as  indicate  an  acute  morbid  blood- state.  But 
this  diagnosis  often  rests  chiefly  on  the  exclusion  of  other  causes ;  if 
these  are  absent,  the  fact  that  neuritis  is  known  to  result  from  obscure 
blood-poisons  may  be  allowed  weight,  especially  if  the  symptoms  begin 
in  the  legs  or  in  aU  parts  of  the  limbs  simultaneously,  and  do  not  affect 
VOL.  I.  12 


178  MULTIPLE    NEURITIS. 

exclusively  at  first  tlie  extensors  of  the  extremities.  Sensory  symptoms 
in  these  cases  at  the  early  stage  often  take  the  form  of  tingling  and 
"  numbness,"  and  the  pains  so  characteristic  of  the  alcoholic  form  are 
frequently  absent,  or  are  represented  only  by  dull  aching.  In  slight 
cases  of  this  type  sensations  of  numbness  and  "  pins  and  needles  "  in 
the  legs,  "with  readiness  of  fatigue  and  loss  of  the  knee-jerk,  may  be 
the  only  symptoms.  The  facts  that  the  nature  of  toxaemic  causes  is  to 
a  large  extent  unknown,  and  that  the  poison  may  probably  be  acquired 
from  without,  make  it  necessary  to  be  prepared  to  fi.nd  this  cause  of 
multiple  neixritis  under  almost  any  circumstances.  Eesidence  abroad 
should  suggest  an  inquiry  for  exposure  to  malarial  influences.  The 
possible  significance  of  tubercle  as  a  cause  should  lead  to  a  careful  exa- 
mination of  the  lungs ;  but  the  possibility  that  phthisis  may  be  an 
effect,  and  not  a  cause,  of  multiple  neuritis  must  be  borne  in  mind. 

Prognosis. — The  danger  to  life  is  in  proportion  to  the  acuteness 
of  the  malady,  to  its  severity  as  measured  by  the  extent  of  the  para- 
lysis, and  to  the  degree  in  which  the  strength  of  the  patient  is 
impaired  by  other  maladies,  or  by  the  cause  of  the  neuritis.  It  is 
important  also  to  remember  that  when  the  affection  has  increased  up 
to  the  time  that  the  patient  comes  under  treatment,  it  generally  does 
not  respond  immediately  to  the  arrest  of  its  cause,  such  as  the  with- 
drawal of  alcohol,  but  continues  to  increase  for  two  or  three  weeks. 
It  is  this  temporary  progressive  tendency  that  constitutes  the  graver 
element  in  the  disease,  and  must  be  taken  into  account  in  every 
initial  prognosis.  Hence  the  forecast  must  be  guarded  in  all  cases 
that  are  severe  when  the  patient  comes  under  treatment,  and  most 
so  if  there  has  been  a  rapid  increase  in  symptoms  that  had  existed  for 
a  long  time  in  slight  degree.  If  the  extensors  of  hands  and  feet  are 
powerless,  and  the  muscles  connecting  the  limbs  with  the  trunk  are 
distinctly  enfeebled,  unless  the  disease  has  clearly  begun  to  lessen, 
there  is  danger  that  a  further  extension  may  involve  the  muscles 
of  respiration;  this  is  especially  great  when  the  muscles  moving 
the  shoulder  are  considerably  weakened.  It  obviously  follows  that 
the  danger  is  still  greater  if  the  muscles  of  the  trunk,  and  especially 
of  the  chest,  have  already  given  evidence  of  changes  in  their  nerves. 
Pain  in  the  trunk  of  the  same  character  as  that  in  the  limbs  is  also 
a  grave  symptom  if  the  motor  power  of  the  limbs  has  become  small, 
or  the  case  is  one  unusually  general  in  its  distribution,  because 
where  the  sensory  fibres  are  suffering  the  motor  fibres  are  in  danger 
also.  Paralysis  of  the  diaphragm  adds  considerably  to  the  danger, 
and  its  power  should  be  carefully  watched  in  the  manner  and  with 
the  precautions  mentioned  in  the  account  of  its  paralysis  (p.  33). 
It  may  become  paralysed  without  the  fact  coming  to  the  conscious- 
ness of  a  patient  lying  quietly  in  bed,  but  a  slight  weakness  of  the 
intercostals  wUl  then  cause  considerable  difficulty  of  breathing  and 
an  accumulation  of  mucus  in  the  lungs,  a  condition  predisposing  to 
definite  bronchitis,  by  which  the  patient  may  be  quickly  suffocated. 


PUOGNOSIS.  179 

The  importance  of  this  point  is  great,  since  respiratory  palsy  is  one  of 
the  most  common  immediate  causes  of  death.  It  is  probable  also 
that  deficient  breathing  power  is  one  of  the  causes,  perhaps  even  the 
chief  cause,  that  lead  to  the  development  of  the  chronic  phthisical 
lung  disease  that  comes  on  in  so  many  prolonged  cases.  Especially 
important,  also,  is  any  indication  that  the  nerves  of  the  heart  are 
suffering.  In  an  acute  increase  due  to  continued  use  of  alcohol  the 
vagus  sometimes  suffers  severely  before  other  nerves,  and  increasing 
frequency  of  the  pulse  is  then  of  most  serious  significance.  The 
prognosis  should  be  guided  by  the  motor  rather  than  by  the  sensory 
symptoms.  The  latter  may  improve  while  the  former  increase  with- 
out lessening  the  gravity  of  the  latter  indication.  Persistent  oedema 
is  of  bad  omen,  and  so  also  are  other  indications  of  considerable  blood- 
change. 

When  once  the  malady  has  become  stationary,  and  its  cause  has 
definitely  ceased,  the  danger  to  life  is  small,  except  in  severe  cases,  in 
which  the  pains  are  intense,  the  patient  helpless,  or  the  heart  is  feeble, 
and  the  pulse  persistently  frequent,  or  there  are  signs  of  lung  disease. 
In  these,  as  in  all  cases,  moreover,  the  danger  is  much  increased  by 
any  indication  that  the  spinal  cord  is  involved.  The  tendency  to  re- 
covery is  far  less  in  the  cord  than  in  the  nerves ;  and  retention  of 
urine  (with  all  its  secondary  consequences),  bedsores,  &c.,  are  prone  to 
occur,  and  to  involve  their  own  danger  even  if  the  disease  in  the  cord 
does  not  increase.  The  prognosis  as  regards  life  in  cases  of  moderate 
severity  depends,  indeed,  as  much  on  complications  as  on  the  state  of 
the  nerves.  Signs  of  chronic  cerebral  meningitis,  of  degeneration  of 
the  walls  of  the  heart,  of  disease  of  the  liver  and  of  the  kidneys,  also 
lessen  the  prospect  of  escape  with  life. 

The  danger  is,  however,  greatest,  and  the  mortality  highest,  in  the 
acute  cases  that  are  due  to  some  toxsemic  state  or  to  cold,  and  it  must 
be  estimated  by  the  rapidity  with  which  the  paralysis  develops  and 
spreads.  Whenever  it  becomes  considerable  in  a  few  days,  the  danger 
of  early  extension  to  the  mechanism  for  respiration  is  very  great.  In 
these  cases,  also,  pneumonia  frequently  occurs,  concomitant  with  the 
early  sjrmptoms,  and  few  cases  thus  complicated  recover. 

In  cases  that  are  not  fatal  the  full  development  of  the  symptoms  is 
followed  by  a  stationary  period,  which  lasts  for  one  or  two  months 
before  improvement  begins,  and  for  this  the  patient  or  friends  should 
be  prepared.  The  course  of  restoration  is  always  extremely  slow ;  the 
muscles  least  affected  regain  power  in  from  two  to  four  months,  but  it  is 
usually  six  months  or  more  from  the  onset  of  improvement  before  the 
.  extensors  are  fairly  strong,  if  their  palsy  was  considerable  or  complete. 
The  intrinsic  muscles  of  the  hand  are  still  longer  in  recovery.  The 
improvement  in  the  nutrition  of  the  muscles  follows  that  in  their 
power,  so  that  they  remain  smaller  than  normal,  long  after  they  have 
regained  strength.  It  may,  indeed,  be  years  before  the  muscles  whose 
size  can  be  well  seen,  such  as  the  abductor  indicis,  are  of  normal  size. 


180  MULTIPLE    NEQRITIS, 

It  is  seldom  that  any  peimanent  weakaess  is  left  unless  tlie  spinal  cord 
is  diseased,  and  when  a  patient  is  distinctly  improving  a  good  ultimate 
prognosis  may  be  given,  provided  there  are  no  complications  to  inter- 
fere with  it.  In  no  case  with  distinct  spinal  symptoms  can  we  feel 
confident  that  restoration  of  power  wUl  be  complete ;  and  in  cases  in 
which  there  are  indications  of  considerable  damage  to  the  cord  it  is 
almost  certain  that  some  lasting  weakness  will  be  left. 

The  electric  irritability  of  the  muscles  and  nerves  may  be  expected 
to  return  to  the  normal  more  slowly  than  their  capacity  of  response  to 
the  will,  but  sooner  than  their  nutrition.  The  conditions  that  are  ob- 
served in  these  cases  are  the  same  as  in  the  isolated  form  of  neuritis, 
in  which,  although  the  primary  change  is  in  the  connective  tissue,  the 
symptoms  depend  on  the  secondary  changes  in  the  nerve-fibres. 
Hence  the  statements  made  on  p.  71  are  equally  apphcable  to  multiple 
neuritis.  The  reactions  often  furnish  important  information,  especially 
soon  after  the  onset ;  the  more  the  irritability  deviates  from  the  normal 
the  greater  will  be  the  subsequent  wasting,  and  the  longer  the  duration 
of  the  palsy.  When  some  faradic  irritabihty  returns  in  both  nerve 
and  muscle  it  may  be  regarded  as  a  sure  indication  of  advancing 
restoration  of  structure,  which  is  certain  to  involve  a  progressive  in- 
crease of  conduction  of  voluntary  impulse,  and  response  to  it.  If  the 
trunk  nerves  are  not  involved,  the  prognosis  in  severe  cases  is  a  little 
better  when  the  arms  are  chiefly  affected  than  when  the  legs  have 
suffered  most. 

The  sensory  disturbance  may  be  expected  to  follow  a  course  on  the 
whole  similar  to  that  of  the  motor  symptoms.  A  diminution  in  the 
spontaneous  pain  may  be  anticipated  sooner  than  in  the  tenderness  of 
the  nerves  and  muscles,  which  usually  lasts,  in  lessening  degree,  until 
the  muscles  have  regained  some  power.  The  extreme  tenderness, 
indeed,  passes  away  with  the  spontaneous  pain,  but  some  degree  of 
over- sensitiveness  continues  for  a  long  time.  Happily  the  tendency  to 
persistent  neuralgia,  seen  after  the  isolated  form,  need  not  be  antici- 
pated after  multiple  neuritis.  We  find  the  explanation  of  this  in  the 
absence  of  the  new  formation  of  connective  tissue  from  the  inflamma- 
tory products,  which  in  the  isolated  form  perpetuate,  by  cicatricial  con- 
traction, the  irritation  to  which  inflammation  gave  rise. 

There  is  little  dift'erence  in  the  prognosis,  as  regards  recovery,  in  the 
different  forms  of  neuritis.  The  anticipations  that  may  be  formed  are 
governed  in  all  by  the  rules  just  mentioned,  and  the  risk  to  life  has 
been  already  considered.  In  aU,  however,  it  may  be  repeated  the 
extent  to  which  the  cause  is  under  control  forms  an  essential  element 
in  the  problem,  and  must  influence  the  conclusion  in  every  case.  The 
prognosis,  however,  is  better  in  the  sensory  than  in  the  motor  form, 
better  when  the  arms  escape  than  when  all  the  limbs  are  involved,  and 
better  in  cases  of  chronic  than  of  acute  onset,  and  better  if  an 
apparently  acute  onset  is  really  such  than  if  it  succeeds  slight  sym- 
ptoms of  longer  duration.     When  the  malady  follows  an  acute  specific 


TRRATMENT.  181 

disease,  the  severity  of  the  latter  is  no  guide  to  the  course  of  its 

sequel ;  severe  neuritis  may  follow  a  mild  attack  of  the  acute  specific. 
Treatment. — The  different  classes  of  multiple  neuritis  here  con- 
si<l<3red  need  treatment  that  in  some  respects  varies,  and  in  others  is  the 
same  in  all  forms.  In  all  it  is  essential  to  discover,  if  possible,  the 
cause  of  the  neuritis,  and  to  remove  it  or  arrest  the  continuance  of  its 
action.  This  obviously  should  be  the  first  consideration  in  cases  due 
to  alcohol,  in  which  it  is  sometimes  of  great  difficulty.  It  is  desirable, 
as  a  rule,  that  alcohol  in  all  forms  should  be  given  up  entirely,  but 
often  this  has  to  be  done  by  degrees,  a  great  reduction  in  amount  pre- 
ceding entire  abstinence  from  it.  With  patients  who  are  "  their  own 
masters  "  the  reduction  is  often  most  difficult  to  secure,  and  it  is  neces- 
sary to  give  a  warning  that  not  only  will  life  be  in  danger,  but  that 
the  pains  will  certainly  increase,  and  continued  indulgence  will  entail 
sufferings  so  great  that  any  sacrifice  would  be  made,  or  deprivation 
endured,  if  their  intensity  could  be  realised.  With  every  female 
drinker,  deception  should  be  considered  not  only  possible  but  probable ; 
those  who  are  under  the  control  of  relations  or  of  a  husband  have 
generally  had  a  long  training  in  duplicity  in  order  to  procure  stimulants, 
and  it  is  easy  for  them  to  continue  it,  even  when  kept  in  bed,  if  they 
are  attended  by  their  own  servants.  These  are  corrupted,  and  alcohol 
is  taken  in  secret  when  the  fact  is  being  stoutly  denied.  If  a  case 
does  not  present  the  arrest  or  improvement  that  might  reasonably  be 
expected  from  the  withdrawal  of  the  cause,  such  continued  drinking 
should  always  be  suspected.  Hence  it  is  of  great  importance  that 
such  patients  should  be,  whenever  practicable,  under  the  immediate 
care  of  trustworthy  trained  nurses,  rather  than  of  their  own  servants 
or  of  relatives  who  may  be  improperly  influenced  or  persuaded ;  if  the 
patient's  means  do  not  permit  this,  removal  to  a  hospital  or  some  public 
institution  is  desirable.  When  the  heart  is  too  weak  to  permit  the 
entire  withdrawal  of  stimulants,  the  amount  taken  must  be  regulated 
solely  by  the  state  of  the  pulse.  In  such  cases  total  abstinence  is 
attended  with  some  danger  of  the  cardiac  failure  which  has  been 
mentioned  as  one  of  the  causes  of  death.  When  the  disease  is  due  to 
cold,  the  patient  should  be  carefully  preserved  from  risk  of  further  ex- 
posure, which  during  the  early  stages  may  convert  a  slight  into  a 
severe  attack.  Even  in  alcoholic  cases  a  chill  will  cause  a  rapid 
increase  in  the  symptoms,  and  both  this  and  fatigue  should  be 
carefully  guarded  against.  In  other  forms,  also,  these  measures, 
adopted  in  the  stage  when  pains  and  tenderness  are  the  only  or  chief 
symptoms,  would  often  prevent  the  further  development  of  the  disease. 
Physical  rest  is  of  great  importance,  and  in  all  cases,  except  the 
slightest,  rest  in  bed  is  desirable  The  excitation  of  the  nerves  caused 
by  movement  tends  to  intensify  the  morbid  process  in  them,  and  what- 
ever produces  pain  has  the  same  effect.  If  the  patient  is  in  bed,  local 
treatment  can  be  more  readily  employed,  and  the  restraint  facihtates  the 
necessary  control  over  habits.      The  feeding  of  the  patient  is  an  impor- 


182 


MULTIPLE    NEUEITIS. 


tant  part  of  treatment,  especially  wheii  the  stomach  has  been  deranged 
by  alcohol.  At  the  outset,  warm  fomentations  should  be  applied  over 
any  tender  nerves.  Care  must  be  taken  in  their  use  over  the  region 
supplied  by  the  affected  nerves,  on  account  of  the  danger  of  vesica- 
tion ;  troublesome  ulcers  may  be  caused  by  even  moderate  degrees  of 
heat,  as  in  the  case  mentioned  on  p.  79.  Such  applications,  and  also 
counter-irritants,  have  less  effect  in  the  parenchymatous  forms  than 
when  there  is  a  tendency  for  the  connective  tissue  and  sheath  to 
suffer,  and  for  their  vessels  to  be  involved  in  the  process.  A  warm 
bath,  for  fifteen  or  twenty  minutes  daily,  is  often  of  service  if  the 
patient  is  able  to  bear  the  necessary  procedures. 

Care  should  be  taken  that  the  patient  does  not  habitually  assume  a 
posture  that  will  promote  the  occurrence  of  deformities.  The  dropping 
of  the  feet  is  the  greatest  danger,  and  should  be  prevented  by  a  large 
and  heavy  sand-bag  about  nine  inches  in  diameter,  which  may  be 
adapted  to  the  position  of  the  feet,  or  by  a  board  placed  across  the 
bed.     Frequently  such  a  boot  as  that  figured  (Fig.  61)  is  of  great 

Fig.  61.* 


service  in  preventing  or  minimising  contraction  of  the  gastrocnemiL 
Another  danger  is  the  habitual  flexion  of  the  knees,  permitting  a 
contraction  of  the  hamstring  muscles,  and  due  to  the  discomfort  that 
is  caused  by  extension  of  the  knees  ;  this  should,  however,  be  insisted 
on,  because  the  initial  discomfort  soon  passes  away  if  extension  is 
adopted  from  the  outset.  The  extension  of  the  knees  involves  that  of 
the  hip-joint,  and  prevents  contraction  of  its  flexors. 

No  drug  has  any  very  marked  influence  on  the  morbid  process.  It 
has  been  seen  that  multiple  neuritis  tends  to  lessen  after  its  cause  has 
ceased  to  act,  and  under  such  conditions  it  is  easy  to  come  to  the  con- 
clusion that  drugs  do  good  which  merely  do  not  hinder.  Mercury, 
which  is  certainly  useful  in  isolated  perineuritis,  has  little  influence 
on  the  parenchymatous  form,  but  it  may  be  tried  in  any  case  in  which 
it  seems  probable  that  the  sheaths  are  primarily  affected,  and  in  which 
pain  or  tenderness  m  the  larger  nerve-trunks  is  a  prominent  sym- 
ptom. During  an  acute  febrile  onset  the  treatment  should  be  that 
*  Por  full  description  of  this  simple  apparatus,  see  '  Clinical  Journal,' March  6th,  1895. 


TitEATMENT.  183 

adapted  to  the  general  state,  and  mnst  difEer  according  to  its  cause. 
In  such  an  onset  of  an  alcoholic  neuritis,  citrate  of  potash,  nitrous 
ether,  and  compound  tincture  of  cinchona  are  suitable,  with  a  little 
digitalis  if  there  is  feebleness  of  the  pulse.  When  due  to  cold,  sali- 
cylate of  soda  may  be  given,  or,  if  the  attack  has  been  predisposed  to 
by  alcohol,  salicylate  of  potash  may  be  substituted  so  as  to  avoid  the 
retention  of  uric  acid  in  the  system  by  the  soda.  The  probability  of  a 
gouty  state  of  the  system  should  be  remembered  in  all  cases  in  which 
there  is  a  history  of  alcoholism.  In  the  toxsemic  forms  in  which 
there  is  a  suspicion  of  a  blood- state  allied  to  that  of  septicaemia,  no 
drug  affords  such  a  prospect  of  influence  as  the  tincture  of  the  per- 
chloride  of  iron  in  full  doses,  20 — 30  minims  three  or  four  times  a 
day.  Afterwards,  when  the  actual  onset  is  over,  and  from  the  first  in 
the  more  chronic  cases,  tonics  answer  best — iron,  quinine,  or  small 
doses  of  strychnine.  Iodide  of  potassium,  for  some  reason,  seems  to 
do  good  only  in  the  chronic  sensory  form.  In  this  also  arsenic  has 
been  found  useful,  but  arsenic  should  be  given  with  caution,  and  in 
small  doses  ;  there  is  some  danger  that  large  doses  may  intensify  the 
malady,  since  polyneuritis  is  one  of  the  effects  of  chronic  poisoning  by 
arsenic,  and  has  actually  been  produced  by  the  medicinal  use  of  the 
metal.  Cod-liver  oil  is  useful  in  the  later  stages,  especially  if  there  is 
impairment  of  general  nutrition.  In  alcoholic  cases  the  addition  of 
cocaine,  -j-V  to  }  grain  to  each  dose  of  whatever  is  taken,  aids  in  lessen- 
ing the  craving  for  stimulant.  When  due  to  malarial  poisoning, 
quinine  may  be  given  freely.  The  complications  of  multiple  neuritis 
do  not  need  other  treatment  than  that  suitable  to  them  when  they 
occur  alone,  nor  do  they  indicate  any  special  modification  in  the  treat- 
ment of  the  disease  of  the  nerves. 

In  most  cases  the  pain  is  such  as  to  render  anodynes  necessary. 
Of  these,  morphia,  by  hypodermic  injection,  is  the  most  effective,  but 
should  be  employed  only  as  a  last  resource,  and  so  regarded  will  not 
often  be  found  necessary.  Its  danger  is  the  readiness  with  which 
those  persons  who  were  previously  alcoholic  become  habituated  to  it. 
It  may  be  given  by  the  mouth  with  less  risk,  but  in  other  drugs  we 
may  find  a  substitute  that  is  effective  in  most  cases.  Injections  of 
cocaine  beneath  the  skin  over  the  seat  of  pain  often  afford  considerable 
relief,  and  cocaine  may  thus  be  given  instead  of  by  the  motith,  securing 
the  double  object  of  lessening  at  once  the  local  pain  and  the  craving 
for  stimulant — which  is  reUeved  almost  as  effectually  when  cocaine  is 
given  by  the  skin  as  by  the  mouth.  Atropine  may  also  be  given 
hypodermically.  In  antipyrin  and  acetanilide  we  have  drugs  of 
another  nature,  that  often  give  great  relief  to  spontaneous  pain. 
Indian  hemp  may  also  be  given  regularly  without  harm,  and  without 
interfering  with  the  occasional  administration  of  other  anodynes  ;  while 
to  procure  sleep,  if  pain  is  slight,  reliance  may  be  placed  on  bromide, 
sulphonal,  trional,  or  chloralamide.  Antipyrin  also  often  gives  a  patient 
•    Tood  night  in  spite  of  some  pain.     When  there  is  meutal  excitement 


184  MULTIPLE    NEURITIS. 

hyoscine  is  useful,  especially  by  hypodermic  injection  in  doses  of  4^  to 
^  or  ^  of  a  grain. 

The  application  of  electricity  to  the  paralysed  muscles  is  important 
in  order  to  maintain  their  nutrition  in  as  good  a  state  as  possible 
duriug  the  process  of  recovery  of  the  nerves.  Only  voltaism  has  the 
power  of  doing  this,  and  should  be  applied  daily,  large  sponge-holders 
being  employed  so  that  the  current  may  reach  as  much  muscular 
tissue  as  possible ;  only  such  a  strength  should  be  employed  as  will 
produce  visible  contraction  and  cause  no  after-pain.  Voluntary  mus- 
cular action  constitutes  a  more  effective  slimvdus  to  nutrition  ;  but  if, 
although  the  muscle  can  be  put  in  action  by  the  will,  there  is  loss  or 
great  diminution  of  faradic  irritability,  or  an  excess  of  voltaic  irri- 
tability shows  that  some  fibres  are  in  an  abnormal  state  in  consequence 
of  the  degeneration  of  their  nerves,  voltaism  may  still  be  applied  with 
advantage.  There  is  no  evidence  that  the  application  of  electricity  to 
the  nerves  has  any  influence  on  their  regeneration.  Shght  sensory  loss 
is  sometimes  lessened  in  the  chronic  stage  by  the  application  of  faradism 
by  the  wire  brush. 

The  tender  limbs  may  be  wrapped  in  cotton  wool,  with  or  without 
a  covering  of  oiled  silk.  Massage  is  of  service  in  the  later  stages  of 
the  disorder  for  its  influence  on  the  nutrition  and  circulation  in  the 
affected  limbs.  An  upward  movement  of  the  pressing  hand  helps  the 
circulation  of  fluid  in  the  vessels  and  in  the  tissues.  It  cannot  be 
borne  during  the  acutely  painful  stage,  and,  indeed,  as  long  as  it  gives 
pain  it  probably  has  more  capacity  for  harm  than  for  good.  In 
the  later  stages  it  helps  to  overcome  the  contracture  of  the  muscles, 
which  shoTild  be  gently  extended  at  the  same  time  as  they  are  rubbed, 
— pressure,  for  instance,  being  made  upon  the  ball  of  the  foot  at  the 
same  time  as  the  calf  muscles  are  rubbed. 

The  very  long  course  of  all  severe  cases  makes  a  heavy  demand  on. 
the  patience  of  the  sufferer  and  the  perseverance  of  the  medical  practi- 
tioner ;  but  the  prolonged  convalescence  has  the  advantage,  in  alcoholic 
cas°s,  of  enabling  a  habit  of  abstinence  to  be  formed.  This,  with  the 
recoLoction  of  what  has  been  endured,  renders  multiple  neuritis  more 
often  a  cure  of  intemperance  than  any  other  of  the  many  maladies  to 
which  alcohol  gives  rise.  During  the  slow  recovery,  the  measures 
above  indicated — tonics,  electricity,  and  massage — should  be  con- 
tinued. As  already  stated,  the  power  of  standing  is  interfered  with 
for  a  time  after  the  muscles  have  regained  adequate  strength,  by  the 
contraction  of  the  gastrocnemii,  causing  a  degree  of  talipes  equinus. 
This  is  often  so  considerable  as  to  suggest  the  desirability  of  dividing 
the  tendo  Achillis,  but  the  operation  is  seldom  if  ever  necessary.  The 
attempt  to  stand  and  walk  constitutes  a  powerfvil  means  of  extension 
of  the  calf  muscles,  before  which  they  soon  yield  sufficiently  to  permit 
the  balance  of  the  body  to  be  maintained,  and  then  progress  becomes 
more  rapid.  The  contraction  at  the  knee  and  hip  is  more  difficult  to 
get  rid  of,  but  generally  yields  in  time  to  persevering  and  gentle  efforts. 


ENDEMIC   NEUfilTlS.  185 


AcTJTB  Multiple  Myositis.    Acute  Polymyositis, 

In  rare  cases  there  occurs  an  acute  interstitial  inflammation  of  the 
muscles,  which  presents  a  general  resemblance  in  distribution,  and  in 
toxic  causation,  to  the  acute  polyneuritis  we  have  just  considered, 
but  differs  in  the  facts  that  the  affection  of  the  nerve- fibres  is  trifling 
and  evidently  subordinate,  and  that  the  subcutaneous  fibrous  tissue  is 
usually  also  inflamed,  causing  oedema  and  erysipelatoid  dermatitis. 
This  is  sometimes  absent  while  the  nerves  are  occasionally  inflamed  in 
greater  degree,  precluding  an  absolute  separation,  although  most  cases 
present  a  sufSciently  uniform  difference  to  make  a  distinctive  type. 
The  malady  is  so  uncommon,  and  the  cases  that  have  been  met  with 
have  varied  so  much  in  severity  and  precise  causation,  that  they  hardly 
do  more  than  illustrate  the  variety  of  special  form  which  may  be  as- 
sumed by  such  toxic  maladies,  and  which  indicate  corresponding 
features  in  the  poison  which  causes  them.  The  symmetry  of  its  mani- 
festations is  conclusive  evidence  that  the  blood  is  its  vehicle,  and  the 
facts  that  a  source  of  definite  poisoning  can  often  be  traced,  while  the 
distal  parts  of  the  limbs  are  commonly  the  first  to  suffer,  constitute  a 
conspicuous  connection  with  polyneuritis. 

Although  not  a  primary  affection  of  the  nervous  system,  its  relations 
justify  its  mention  here,  especially  since  its  features  have  enough  in 
common  with  the  acute  form  of  midtiple  neuritis  to  involve  some  risk 
of  confusion  in  diagnosis. 


ENDEMIC  NEITEITIS. 
Malaeial  Neuritis. 

The  clear  evidence  that  has  accumulated,  showing  the  dependence 
of  multiple  neuritis  on  toxic  blood-states,  some  connected  with  an 
organismal  virus,  prepares  us  to  find  that  it  often  results  from  the  pro- 
ducts of  the  growth  of  organisms  of  endemic  character,  such  as  cause 
the  malarial  fevers  of  which  we  are  able  to  discern  a  variety  of  forms. 
Conspicuous  among  them  is  tbe  malady  known  generally  by  the  name 
of  "  beri-beri,"  but  a  simpler  form  of  neuritis  palsy  is  more  common, 
although  absolutely  infrequent.  Various  forms,  however,  result  from 
the  untypical  varieties  of  malarial  disorder,  and  their  occurrence, 
causation,  and  relations  are  stUl  only  imperfectly  discerned,  met  with, 
as  they  are,  chiefly  in  tropical  coim tries. 

The  characteristic  form  involves  chiefly  the  motor  nerves,  usually  of 
the  legs,  and  is  met  with  espc'  ially  in  India  and  on  the  coasts  of 
Africa.  The  arms  suffer  only  in  severe  and  prolonged  cases.  It  is 
usually  subacute  ia  course,  and  the  resulting  disability  entails  early 
removal  from  the  causal  conditions,  which  lessens  much  the  danger  to 


186  MULTIPLE    NEURITIS. 

life  that  is  involved.  Socondary  contracture  in  the  mnscles  is  especi- 
ally common,  and  constitutes  a  troublesome  sequel,  prolonging  for 
months  the  inability  to  walk,  unless  prevented  by  the  means  described 
elsewiiere. 

Sensory  symptoms  of  various  characters  may  be  the  leading  features 
in  other  cases  in  which  the  organistns  are  peculiar  in  their  nature,  and 
these  give  rise  to  symptoms  which  are  prone  to  set  up  special  diffi- 
culties in  diagnosis,  unless  their  probable  nature  is  suspected,  and  the 
significance  of  the  exposure  to  the  cause  is  perceived,  and  receives  due 
weight. 

Beri-beei. 

Beri-beri,  the  Kak-ke  of  Japan,  is  also  known  by  various  popular 
names  in  the  several  countries  of  which  it  is  native.*  It  is  a  wide- 
spread disease,  passing  by  a  contagion  that  is  readily  conveyed  and 
propagated,  having  apparently  its  chief  homes  in  Japan,  the  Eastern 
Archipelago,  India,  New  Zealand,  Ceylon,  the  South  Pacific  Islands, 
and  the  coast  of  Brazil.  It  is  especially  prevalent  in  the  Dutch  East 
Indies,  among  the  soldiers  and  in  the  prisons,  and  this  led  to 
its  systematic  investigation  under  the  direction  of  the  Netherlands 
Government.  Through  this  our  knowledge  of  the  malady  was  much 
increased,  especially  by  the  investigations  of  Pekelharing  and  Winkler.f 
who  had  a  large  number  of  cases  under  their  observation  both  during 
life  and  after  death.  Still  more  recently  a  troublesome  outbreak 
occurred  at  a  lunatic  asylum  at  Dublin.  Opinion  as  to  its  nature 
and  cause  has  varied  much,  and  still  is  far  from  uniform  among  those 
who  have  studied  it ;  but  there  is  a  strong  preponderance  of  evidence 
that  it  depends  on  a  specific  organism,  and  that  symmetrical  peripheral 
neuritis  is  the  common  effect  of  the  virus,  and  the  mechanism  by 
which  its  chief  symptoms  are  produced.  The  organisms  found  J  are 
in  the  form  of  rods  and  cocci,  but  it  is  probable  that  these  are  only 

*  Beri-beri  is  probably  a  inodification  of  the  Cingalese  name  for  the  disease,  ball r- 
bari  =  extreme  weakness,  Kak-ke  is  the  old  Chinese  name  for  it,  by  which  it  is 
mentioned  in  their  medical  works  since  200  B.C.;  it  is  derived  from  two  words, 
meanino'  "legs"  and  "disease."  The  affection  disappeared  from  China  two  cen- 
turies ago,  remaining  in  Japan,  where  it  continues  a  most  serious  endemic  malady. 

f  Pekelliaring  and  Winkler,  'Onderzoek  naar  den  aarden  de  oorzaak  der  Beri- 
beri,' Utreclit  (sep.  publication),  1889,  analysed  in  the  '  Centralbl.  f.  Nervenkr.,' 
1889;  also  '  Weekblad  f.  Nederl.  Geneesk.,'  1888,  and  *  Deut.  med.  Wochenschr.,' 
1888,  No.  30.  Other  important  contributions  to  the  subject  are  by  Biilz, 
•Zeitschr.  f.  kl.  Med.,'  1881  j  Scheube,  ib.,  1882;  Weintraub,  *Wien.  med. 
Wochenschr.,'  1888,  Nos.  23—44;  Van  Eecke,  'Tijdschr.  v,  Nederl.  Indre,'  1887, 
p.  71;  Wernich,  *  Virchow's  Archiv,'  Bd.  Ixxi ;  Miura,  •  Virchow's  Arcliiv,' 
Bd.  cxi  and  cxiv,  whose  conclusions,  however,  differ  from  those  of  most  investi- 
gators; Seguin,  'Phil.  Med.  and  Surg.  Rep.,'  1888;  and  Sprir.gthorpe, 'Australian 
Med.  Journal,'  1889.  Balz,  in  1881,  expressed  the  opinion  that  the  disease  was 
a  specific  "  panneuritis." 

t  First  described  by  Balz  and  Scheube,  and  since  by  Pekelharing  and  Winklpr, 
Eigkman,  Weintraub,  Springthorpe,  and  others. 


BERI-BERI.  187 

different  stages  in  the  development  of  the  same  species.  They  have 
been  cultivated,  and  peripheral  nem-itis  of  nearly  the  same  distribution 
as  in  beri-beri  has  been  produced  by  their  inoculation.  Repeated  in- 
oculations are,  however,  necessary  for  this  result  to  be  produced ;  and 
hence,  from  this  and  the  phenomena  of  the  disease,  it  is  assumed  to 
be  not  a  simple  infectious  malady,  capable  of  being  induced  by  a 
single  exposure  to  its  cause,  but  one  in  which  repeated  opportunities 
for  infection  are  necessary.  In  harmony  with  this  are  the  facts  that 
sufferers  acquire  the  disease  from  residence  in  certain  infected  houses, 
or  places  in  which  it  is  supposed  that  the  soil  is  saturated  with  the 
organisms ;  that  they  may  rapidly  recover  on  removal  to  a  district 
that  is  free,  and  relapse  only  on  returning  to  an  infected  place ;  that 
it  prevails  where  persons  are  gathered  together,  as  m  barracks  and 
prisons;  and  that  the  air  of  these  places  is  found  to  contain  the 
organisms  which,  collected  from  it,  will  cause  the  disease  in  animals, 
from  whom  it  is  transmissible  to  others  by  further  inoculation.  The 
serious  extent  to  which  it  may  spread  among  those  who  live  together 
under  conditions  favouring  its  extension,  is  shown  by  the  instance  of 
a  ship  ai'riving  in  Japan  from  New  Zealand  after  a  voyage  of  272 
days.  The  disease  spread  on  board  so  rapidly  that  altogether  169 
cases  occurred,  with  twenty-five  deaths.  In  1878  no  less  than  38  per 
cent,  of  Japanese  soldiers  were  affected.  It  is  probable  that  the  in- 
halation of  dried  organisms  floating  in  the  air  as  part  of  "  dust,"  is 
the  chief  way  in  which  the  disease  is  propagated.  By  some  it  has 
been  thought  that  a  nitrogenous  diet  induces  or  predisposes  to  the 
disease,  and  facts  apparently  supporting  the  opinion  have  been 
adduced ;  an  exclusive  fish  diet  has  been  thought  to  cause  it,  and  so 
also  has  a  rice  diet.  A  remarkable  outbreak  in  Manila  in  1880  followed 
a  period  of  rice-eating  during  a  cholera  epidemic.  These  influences 
probably  merely  produce  susceptibility.* 

It  is  a  remarkable  fact  that  Europeans  seldom  suffer.  Males  are 
more  liable  to  the  disease  than  females,  and  it  is  chiefly  prevalent 
during  the  hot  season. 

Symptoms. — Peripheral  symmetrical  neuritis  is  a  constant  feature 
of  the  malady,  which  is  usually  chronic  in  course  and  gradual  in 
onset,  but  prone  to  undergo  acute  exacerbations.  These  have  been 
generally  regarded  as  acute  forms  of  the  disease,  but  it  is  said  by 
Pekelharing  that  the  symptoms  of  neuritis  may  always  be  discovered 
before  the  onset  of  the  definite  symptoms,  and  this  when  the  patient 
ig  ignorant  of  their  existence  and  feels  quite  well.  Most  observers 
have  failed  to  recognise  this,  and  it  is  probably  true  only  under 
certain  conditions,  and  persons  suddenly  exposed  to  an  intense  infec- 
tion may  suffer  acutely  from  the  very  commencement.     Occasionally 

*  Putnam  ('  liostou  Med.  and  Surg.  Journal,'  1890)  and  Birge,  in  tlie  same 
periodicMl,  describe  a  form  of  neuritis  very  lilte  beri-beri  occurring  in  men  long  afc 
eea  eng.nged  in  fishing,  with  poor  food,  lu  some  of  the  cases  scurvy  waa  also 
present. 


188  MULTIPLE    NEURITIS. 

the  mailady  develops  witli  exti'eme  rapidity  and  severity.  On  the  other 
hand,  many  cases  are  chronic  throughout,  and  last  for  months.  The 
leading  symptoms  are  those  of  multiple  neuritis  (chiefly  affecting  the 
legs  and  the  cardiac  branches  of  the  vagus),  dropsy,  and  symptoms  of 
cardiac  failure.  The  amount  of  urine  is  generally  lessened,  and  its 
secretion  may  be  almost  suppressed  in  acute  cases.  A  "  critical " 
increase  in  the  secretion  may  mark  the  commencement  of  improve- 
ment. It  is  unchanged  in  character,  and  does  not  contain  albumen. 
The  dropsy  is  a  very  variable  symptom,  and  this  has  led  to  the  dis- 
tinction of  two  forms,  the  dry  and  the  dropsical.  Pekelharing  and 
Winkler,  however,  found  some  effusion  of  fluid  almost  invariably  after 
death,  and  it  is  probable  that  its  conspicuous  presence  or  absence 
depends  chiefly  on  the  state  of  the  heart  (conditioned  by  that  of  its 
nerves),  and  partly  on  the  trophic  and  vaso-motor  disturbances  due  to 
the  local  neuritis.  One  very  constant  symptom  is  said  to  be  a  dis- 
agreeable epigastric  sensation,  variously  described  as  pain,  drag- 
ging, &c.,  sometimes  very  severe.* 

The  earliest  symptoms  are  a  change  in  the  electrical  excitability  of 
the  peroneal  nerves  and  the  flexors  of  the  ankles  (which  suffer  most 
throughout) — a  slight  degree  of  the  reaction  of  degeneration,  quantita- 
tive and  often  qualitative.     These  are  often  to  be  found  before  there 
are  any  subjective  symptoms,  which  begin  as  a  sense  of  heaviness  of 
the  legs,  readiness  of  fatigue,  dyssesthesiae  and  diminution  of  tactile 
sensibility  in  the  lower  limbs,  palpitation  and  undue  excitability  of  the 
heart.     The  electrical  changes  may  be  met  with  in  slight  cases  which 
proceed  no  further,t  and  have  shown  that  some  patients,  supposed  to 
be  shamming,  were  real  sufferers.     To  these  subjective  symptoms  are 
added  other  objective  signs, — oedema  along  the  edge  of  the  tibiae,  a 
peculiar  pasty  and  stiff  aspect  in  the  face,  an  increase  in  the  cardiac 
dulness  to  the  right,  roughness  of  the  first  and  accentuation  of  the 
pulmonary  second  sound.     These  symptoms  may  increase  slowly,  or 
rapidly  in  the  form  of  an  acute  stage.     The  degenerative  reaction 
becomes  complete  in  the  muscles  first  affected,  and  they  become  para- 
lysed and  undergo  the  characteristic  atrophy,  while  other  nerves  and 
muscles  progressively  suffer  in  the  same  manner, — the  calf  muscles,  the 
extensors  of  the  knee,  the  adductors  of  the  thigh,  and,  lastly,  the 
flexors  of  the  thigh  and  the  abductors  of  the  hip.     In  the  trunk  the 
abdominal  muscles  and  intercostals  may  be  involved,  and  in  severe 
cases  the  arms  are  paralysed,  first  the  extensors  of  the  wrist  and 
fingers,  later  the  flexors,  and  sometimes  most  of  the  muscles,  may  be 
so  atrophied  as  almost  to  disappear.     The  face  often  suffers:   the 
diaphragm  may  become  paralysed,  and  also  the  larynx  (the  inferior 
before  the  superior  laryngeal  nerve)  ;  while  grave  cardiac  weakness 
and  increasing  dilatation  of  the  heart  testify  to  the  serious  implica- 
tion of  the  cardiac  branches  of  the  vagus.     Simultaneously,  sensory 

*  Von  Tuzelinaini,  '  Lancet,'  1894,  vol.  ii,  p.  1467. 
t  Pekelharing  aiul  Winkler,  Eigkman  (1889),  &C. 


BERI-BERI.  189 

•yiiiptoms  develop,  corresponding  in  distribution  to  tlie  more  severe 
motor  symptoms ;  sensibility  to  touch  is  lessened  or  lost  (first «:  n  the 
inner  side  of  the  lower  limb),  while  that  to  pain  usually  remains,  and 
sometimes  is  augmented  to  the  degree  that  constitutes  what  has  been 
termed  "  anaesthesia  dolorosa."  The  temperature- sense  may  be 
diminished  to  either  heat  or  cold  or  both  in  various  parts,  and  there 
is  often  considerable  loss  of  cutaneous  sensitiveness  to  faradism. 
There  may  be  tingling,  formication,  and  other  dyssesthesise,  together 
with  tenderness  of  the  nerves  and  muscles,  but  far  less  in  degree  than 
in  most  forms  of  polyneuritis,  and  chiefly  marked  in  the  early  stage  of 
the  disease. 

1'he  oedema  that  is  so  common  begins  in  the  legs,  usually  spreads 
widely,  and  involves  not  only  the  subcutaneous  tissue  but  the  cavities 
of  the  peritoneum,  pleura,  and  pericardium :  in  the  last  it  seriously 
impedes  the  action  of  the  already  dilated  and  feeble  heart.  It  is  to 
cardiac  failure  that  death  is  commonly  due ;  the  other  chief  cause 
is  failure  of  the  respiratory  muscles,  aided  generally  by  effusion  into 
the  pleural  cavities,  and  accompanied  by  the  indications  of  cardiac 
weakness,  and  of  dilatation  of  the  right  side  of  the  heart.  From  this 
cause  the  cardiac  dulness  sometimes  increases  rapidly,  even  in  a  few 
hours.  The  oedema  is  usually  attended  by  increasing  anaemia,  which 
doubtless  facilitates  its  occurrence.  Thes^'  symptoms  are  always  later 
in  development  than  are  those  due  directly  to  the  neuritis.  In  the 
most  acute  cases  the  definite  symptoms  take  commonly  six  or  eight 
weeks  to  run  their  course,  and  the  usual  duration  of  the  malady  is 
several  months.  Death  from  the  heart  failure  is  usually  prolonged, 
and  accompanied  by  much  distress.  Occasionally  a  very  acute  form 
prevails,  in  which  rapid  failure  of  the  heart  and  various  effusions 
lead  to  death  in  about  a  fortnight,  and  cause  a  mortality  of  60  and  70 
per  cent. 

Pathological  Anatomy. — The  only  constant  changes  are  in  the  nerves, 
but  some  serous  effusion  is  almost  invariable.  Thus  Pekelharing  and 
Winkler  found  an  excess  of  fluid  in  the  cellular  tissue  in  88  out  of  85 
cases — about  97  per  cent.  The  pericardial  fluid  was  increased  in  67 
per  cent,  of  the  cases,  in  about  23  per  cent,  there  was  hydrothorax, 
and  ascites  in  14  per  cent. 

The  changes  in  the  nerves  are  often  indistinct  to  the  naked  eye, 
although  always  conspicuous  Tinder  the  microscope.  They  are  met 
with  in  both  motor  and  sensory  branches,  and,  in  advanced  cases,  in 
the  nerves  of  the  vessels.  They  are  always  greatest  at  the  terminal 
portions,  lessening  upwards,  while  the  nerve-roots  and  spinal  cord 
are  always  normal,  with  the  doubtful  exception  of  vacuolation  of  the 
motor  cells,*  and  some  degeneration  of  the  fibres  of  the  posterior 
column  in  a  few  cases. f  The  first  change  is  an  increase  in  the  seg- 
mental nuclei,  shrinkage  of  the  medullary  sheath  at  the  nodes,  and  a 

*  Mi  lira,  loc.  cit. 

t  Van  Eecke,  loc.  cit. 


190  MULTIPLE    NEUKITIS, 

peculiar  "lumpy"  degeneration  of  tlie  medulla,  sometimes  replaced 
by  a  "foam-like"  appearance  in  it,  concealing  the  axis- cylinder. 
Both  changes  are  often  accompanied  by  the  formation  of  granule 
corpuscles.  These  slighter  changes  are  found  chiefly  where  the  lesion 
is  commencing,  and  especially  in  the  vagus  and  phrenic  nerves,  in 
which,  necessarily,  a  considerable  degree  of  change  is  incompatible 
with  life.  They  have  been  seen  in  the  branches  within  the  heart,  in 
some  of  the  fibres  of  the  trunk,  and  in  the  laryngeal  nerves.  Where 
the  change  in  the  nerves  is  more  advanced,  the  white  substance  is 
entirely  broken  up,  and  the  axis -cylinder  presents  segmentation  or 
irregular  swellings,  or  has  disappeared,  and  the  increase  in  the  nuclei 
of  the  neurilemma  sheath  is  still  greater  than  before.  The  blood- 
vessels of  the  nerves  are  but  little  changed  in  the  early  stage  ;  after  a 
time  they  may  present  some  thickening  of  the  wall,  accompanied  by  a 
general  increase  in  the  amount  of  the  interstitial  tissue ;  but,  as  a  rule, 
there  is  an  absence  of  any  signs  of  interstitial  inflammation — ^the 
changes  are  essentially  parenchymatous. 

In  the  muscles  there  may  be  at  first  a  cloudy  opacity  with  indis- 
tinctness of  the  transverse  strise,  going  on  to  granular  degeneration ; 
or  the  fibres  may  simply  become  narrow,  preserving  their  striation. 
The  nuclei  of  the  fibre-sheath  are  always  increased  in  number,  but 
the  interstitial  tissue  is  generally  but  little  altered. 

Pathology. — The  characteristic  organisms  have  been  invariably  found 
in  the  blood,  and,  what  is  a  remarkable  instance  of  their  slow  action, 
and  of  the  cumulative  influence  needed  to  generate  the  malady, 
Pekelharing  and  Winkler  *  found  them  in  the  blood  of  aU  the 
inhabitants  of  an  intensely  infected  place,  whether  these  had  or 
had  not  symptoms  of  the  disease,  and  failed  to  find  them  in  the  blood 
of  persons  living  in  places  free  from  infection.  Cultures  from  the 
blood  reproduce  the  malady,  and  so  do  cultures  from  the  animals 
thus  infected.  The  organisms  have  not  been  found  in  special  associa- 
tion with  the  morbid  process  in  the  nerves.  It  thus  appears  that  the 
malady  bears  a  close  resemblance,  in  its  essential  features,  to  cases  of 
multiple  neuritis  due  to  a  chronic  blood-poison  such  as  alcohol,  and 
still  more  to  that  which,  as  we  shall  see,  is  presented  by  chronic 
arsenical  poisoning.  There  is  a  slow  accumulation  in  the  blood  of  a 
virus  that  has  a  specific  action  on  the  peripheral  nerves,  and  like  some 
others,  first  and  chiefly  on  the  nerves  of  the  legs,  but  with  a  greater 
affinity  for  the  vagus,  especially  for  its  cardiac  branches,  than  most 
others.  Although  invariably  associated  with  the  presence  in  the  blood 
of  a  special  organism,  the  absence  of  this  in  the  vicinity  of  the  lesion, 
taken  in  conjunction  with  modern  researches  in  bacteriology,  makes 
it  probable  that  the  virus  is  a  chemical  substance  produced  by  the 
organism  in  its  growth.  This  brings  the  disease  into. close  afiinity 
with  some  other  forms  of  multiple  neuritis,  on  which  it  throws  suffi- 

*  Also  Ogata  (1888)  and  others.  They  have  also  been  found  sparingly  in  the 
organs,  even  in  the  spinal  cord,  which  is  commonly  f'-ee  from  disease. 


BERl-BEEI.  19] 

cient  light  to  justify  ttis  somewhat  full  account  of  a  malady  that  ia 
not  likely  practically  to  concern  many  readers.  If  we  regard,  as  appa- 
rently we  must,  the  cardiac  failure  as  the  result  of  the  cardiac  neuritis, 
and  the  dropsical  effusion  as  the  combined  result  of  this  and  the  local 
trophic  and  vaso-motor  influence  of  the  neuritis  (to  which,  perhaps, 
the  change  in  the  blood  should  be  added),  we  have  a  remarkable  illus- 
tration of  the  extent,  variety,  and  degree  of  the  secondary  processes  of 
disease  which  multiple  neuritis  may  produce. 

The  Diagnosis  can  only  be  a  matter  of  diflB.culty  in  a  very  early 
case,  or  in  a  patient  who  is  not  known  to  have  been  exposed  to 
the  infection,  or  in  consequence  of  ignorance  of  the  disease.  Doubt 
may  exist,  however,  whether  in  a  given  case  the  malady  is  alcoholic 
neuritis  or  beri-beri.  The  fonner  is  probable  if  the  arms  are  involved 
at  the  same  time  as  the  legs,  still  more  if  they  suffer  first ;  also  if 
pains  are  severe,  oedema  absent,  and  the  heart  unaffected.  The  con- 
verse would  make  the  endemic  malady  probable.  Beri-beri  has  cer- 
tainly been  intensified  by  alcohol  in  some  cases,  and  the  symptoms 
then  presented  are  a  combination  of  the  two, — pains,  associated  with 
the  weakness  and  enlargement  of  the  heart  and  the  widespread 
tendency  to  dropsical  effusion  met  with  m  beri-beri.  In  most  cases 
some  other  indication  of  the  influence  of  alcohol,  especially  enlarge- 
ment of  the  liver,  will  assist  the  diagnosis.  From  other  forms  of 
multiple  neuritis  it  is  unlikely  that  the  diagnosis  would  have  to  be 
made.  From  affections  of  the  spinal  cord  the  distinction  (chiefly 
needed  in  the  chronic  "dry"  variety)  depends  on  the  points  already 
mentioned  in  the  account  of  multiple  neuritis.  Acute  cases  with 
much  oedema  and  weakness  of  the  heart,  and  enlargement  of  the  right 
side,  may  be  mistaken  for  primary  heart  disease ;  but  if  the  symptoms 
of  beri-beri  are  known,  the  affection  may  be  recognised  by  the  absence 
of  any  indications  of  primary  valvular  disease ;  while  an  examination 
of  the  legs  will  reveal  the  weakness  of  the  flexors  of  the  foot  and 
lessened  tactile  sensibility  in  the  skin  over  them. 

Treatment. — Beri-beri  is  one  of  those  diseases  for  which  it  is 
probable  that  a  method  of  at  least  prophylactic  inoculation  will  be 
discovered  in  the  futiire.  At  present  the  destruction  of  the  organisms 
by  disinfecting  agents,  or,  better,  the  removal  of  a  person  to  an  unin- 
fected district,  constitutes  the  only  means  of  preserving  him  from  the 
continued  entrance  of  the  poison — a  measure  essential  for  successful 
treatment,  and,  in  many  cases,  all  that  is  really  necessary.  The 
limits  of  disinfection  are,  however,  reached  with  the  dwelling-house ; 
and  the  great  difficulty  presented  by  the  disease  is  the  manner  in 
which  whole  districts  are  capable  of  giving  rise  to  it,  probably  by 
the  infection  of  the  soil,  which  cannot  be  rendered  innocuous. 
However,  even  within  the  narrow  limits  of  personal  use,  disinfection 
has  achieved  some  remarkable  results,  and  in  few  diseases  has  the 
light  of  pathology  given  more  useful  guidance  to  the  hand  of  the 
practitioner 


192  MULTIPLE   NEURITIS. 

In  most  respects  tlie  treatment  of  the  symptoms  of  beri-beri  does 
not  differ  from  that  of  alcoholic  neuritis.  A  special  point,  however, 
is  that  the  strengthening  of  the  heart  by  digitalis  or  strophanthus  ia 
more  often  of  urgent  importance,  and  requires  greater  care  lest  the 
feeble  fibres  be  overtaxed.  Small  doses  should  therefore  be  given — 
three  or  at  most  five  minims  of  the  tincture  of  digitalis.  Bodily 
rest  is  of  paramount  importance  to  the  weak  organ,  in  order  that 
there  may  not  be  any  demand  on  its  energies  for  other  work  than 
that  involved  in  the  maintenance  of  what  may  be  termed  static 
nutrition,  and  that  it  may  be  spared  the  increased  work  necessi- 
tated by  muscular  exertion.  Strychnia,  quinine,  &c.,  may  do  good, 
but  their  use  in  this  disease  does  not  differ  from  that  in  the  more 
common  forms  of  multiple  neuritis. 


Lepeotjs  !N'etjritis. 

Far  removed  as  leprosy  may  seem  to  be  from  the  malady  last 
described,  both  by  its  general  character  and  extremely  chronic  course, 
it  is  certainly  also  an  endemic  disease;   its  cause  must  be    sought 
in  local  influences ;  and  it  agrees  with  beri-beri  in  the  nature  of  those 
influences.     Since  the   researches   of    Carter,   Hansen,   and   Neisser 
showed  the  constant  presence  of   a  specific  bacillus  in  all  the  new 
formations  met  with  in  the  disease,  their  observations  have  received 
abundant  corroboration  ;  the  disease  was  indeed  one  of  the  first  to  be 
proved  to  depend  on  living  organisms.     The  virus  resembles  that  of 
beri-beri  in  being  transmissible  from  individual  to  individual,  but 
differs  in  its  capability  of  passage  by  inheritance,  in  the  less  readiness 
with  which  it  passes  from  person  to  person,  and  in  the  extreme  slow- 
ness with  which  it  commonly  develops  to  the   degree  necessary  to 
cause  symptoms.     Tears  may  elapse  after  the  last  possible  exposure, 
before  distinct  symptoms  of  leprosy  appear.     In  the  case  from  which 
Fig.  63  was  taken  the  sufferer  was  a  European  of  healthy  parentage, 
>.ho  could  not  have  been  infected  within  ten  years  of  the  first  sym- 
ptoms.    In   one  case   of   infection  from  adult  to  adult,  nine   years 
elapsed  after  the  death  of  the  leper  before  the  man  who  had  lived 
with  him  began  to  suffer.      When,  indeed,  a  large  quantity  of   the 
virus   is   received,  the   malady   develops   much   more    rapidly.     But 
leprosy  differs  from  beri-beri,  and  from  most  other  forms  of  multiple 
neuritis,  in  the  fact  that  although  inflammation  of   many  nerves  is 
frequently  a  part  of   the   disease,  it  is  not  an  essential  part  or  a 
constant  feature  of   the  affection.      On  the  contrary,  it  is  only  one 
of  an  extensive  series  of  lesions,  inconstant,  not  only  in  its  occur- 
rence, but  in  its  seat  and  extent.     It  characterises  a  special  variety 
Df  the  disease,  the  "anaesthetic  leprosy,"  in  which  not  only  areas  of 
the  skin  become  insensitive,  but  the  ends  of  the  fingers  may  suffer 
such  an  impairment  of  their  nutrition  that  they  are  lost.     Moreover, 


LEPKOUS    NEUiaTIS.  193 

as  we  have  seen  in  the  introductory  section,  the  want  of  strict  sym- 
metry is  associated  with  an  important  difference  from  the  symmetrical 
forms — the  neuritis  is  not  a  parenchymatous  or  degenerative  form, 
but  is  a  periaeuritis  and  interstitial  neuritis,  and  the  damage  to  the 
fibres  is  secondary.  This  change  in  the  connective  tissue  is  asso- 
ciated with  the  actual  presence  of  the  organisms  in  it.  The  neuritis 
thus  seems  to  be  due  to  the  direct  action  of  the  bacilli  on  the  affected 
tissues,  and  not,  as  in  beri-beri,  to  a  virus  circulating  in  the  blood — 
not  to  a  product  of  the  organisms,  but  to  the  organisms  themselves. 
This  fact  enables  us  to  understand  better  its  peculiar  features,  and 
especially  its  irregular  distribution;  the  inflammation  occurs  where 
the  organisms  happen  to  fix  themselves  in  the  connective  tissue,  and 
although  certain  common  conditions  on  the  two  sides  may  determine 
a  partial  correspondence  in  the  nerves  affected,  there  is  not  the  sym- 
metry that  results  from  an  action  on  like  structures  on  the  two  sides, 
equally  accessible  to  the  virus.  In  this,  and  in  its  connective-tissue 
seat,  the  neuritis  of  leprosy  resembles  more  the  isolated  neuritis  of 
syphilis,  rather  than  the  common  varieties  of  the  multiple  form.* 

The  symptoms  of  the  malady  are  extensive  and  various,  but  only 
those  that  consist  in  derangement  of  the  functions  of  the  nervous 
system  concern  us  here,  and  it  is  alike  needless  and  impracticable 
to  describe  the  others.  The  nervous  symptoms  of  leprosy  depend 
almost  exclusively  on  inflammation  of  the  nerves.  These  symptoms  are 
muscular  wasting  and  ansesthesia,  greatest  in  degree  towards  the  ex- 
tremities of  the  limbs,  but  more  or  less  irregular  in  situation,  and  in 
the  region  of  the  distribution  of  nerve-trunks,  which,  if  accessible,  are 
obviously  diseased.  The  symptoms  are  not  confined  to  the  limbs,  but 
are  also  met  with  in  the  face,  where  an  affection  of  the  fifth  nerve  may 
cause  ansesthesia,  and  movement  may  suffer  from  involvement  of  the 
trunk  of  the  facial  nerve,  so  that  the  eyelids  cannot  be  completely 
closed.  The  form  of  sensibility  that  is  impaired  also  varies ;  sometimes 
touch,  sometimes  pain  is  lessened  in  greater  degree,  but  usually  all 
forms  are  implicated  to  some  extent.  The  legs  often  suffer  before  the 
arms,  but  a  special  order  of  affection  cannot  be  distiaguished.  There 
may  be  unpleasant  tiagling  sensations,  but  pains  are  usually  trifling, 
perhaps  because  the  process  is  extremely  chi-onic.  In  some  cases  there 
may  be  severe  pain  in  the  position  of  the  nerves  or  deeply  seated  in 
the  limbs.  The  inequality  of  the  affection  of  the  fibres  of  the  same 
nerve  is  shown  by  the  fact  that  the  knee-jerk  may  persist  although 
the  extensors  of  the  knee  are  partially  paralysed.  Fig.  62  shows  the 
distribution  of  anaesthesia  in  the  hands  in  a  well-marked  example 
of  this  disease  which  came  under  my  observation  some  years  ago. 
The  patient  was  a  Creole  of  Mauritius,  who  had  come  to  England 
in  early  childhood,  and  presented  the  first  distinct  symptoms  at 

*  Gerlacb  (•  Virch.  Arch.,'  cxxv,  *Neur.  Cent.,'  1891,  p.  464)  says  infection  is  from 
skin,  branches  of  nerves  being  then  affected  and  finally  the  nerve-trunks,  so  that 
skin  may  be  seen  infiltrated  when  nerves  still  remain  free. 

VOL.  I.  13 


194 


MULTIPLE    NEUEITIS. 


twelve  years  of  age — changes  in  fhe  pigmentation  of  tlie  skin,  auses- 


thesia,  and  muscular  wasting. 


They  steadily  increased  during  the 
next  four  years,  the  anaesthesia 
extending  each  half-year  from 
one  finger  to  another.  There 
was  also  patchy  anaesthesia  of 
the  legs  below  the  knee.  As 
the  figure  shows,  the  loss  of 
sensation  does  not  correspond 
in  area  to  nerve  distribution. 
This  depends  on  the  fact  that 
even  when  all  the  nerves,  in- 


PlQ-.  62. — Distribution  of  anaesthesia  in  a 
case  of  leprosy.  The  black  areas  indicate 
the  loss. 


eluding  those  of  the  fingers,  are 
diseased,  all  the  fibres  may  not 
be  destroyed  in  any  one  nerve, 
so  that  some  sensibility  exists 
in  each  nerve  area.  Various 
perversions  of  sensibility,  spon- 
taneous "  dyssesthesise,"  ting- 
ling, formication,  and  the  like, 
may  precede  the  loss.  The 
latter  usually  occurs  gradually, 
but  sometimes  so  suddenly  as 
to  suggest  some  secondary 
vascular  lesion,  haemorrhage  or 
thrombosis,  in  the  vessels  of  the  nerve  that  is  the  seat  of  the  process 
— a  possible  result  in  its  early  and  most  active  stage.  It  must  be 
remembered  that  in  all  inflammations  of  the  sheath  and  interstitial 
tissues  this  proceeds  to  a  considerable  degree  before  the  fibres  of  the 
nerve  suffer,  and  thus  sudden  loss  of  sensibility  does  not  show  that 
the  nerve  was  previously  healthy.  This  occasionally  sudden  onset  is 
of  much  practical  importance ;  one  case  was  sent  to  me  from  a  place 
in  which  the  disease  is  endemic,  by  a  doctor  well  acquainted  with  it, 
who  had  not  suspected  leprosy  because  the  anaesthesia  came  on  almost 
suddenly. 

Accompanying  anaesthesia  there  is  usually  some  muscular  wasting, 
which  may  be  great.  In  the  case  figured  it  was  considerable,  although 
only  in  the  small  muscles  of  the  hands  was  it  comparable  to  that  of 
progressive  muscular  atrophy.  The  electrical  excitabihty  of  the  mus- 
cles was  greatly  lowered  to  each  current,  and  this  is  perhaps  the  most 
common  condition  ;  either  the  damage  to  the  nerves  is  so  chronic  that 
the  fibres  undergo  slow  changes  paH  passu  with  those  in  the  nerves  and 
their  endings,  or  the  patient  comes  under  observation  at  a  late  stage 
in  the  local  affection,  at  which  the  changes  present  in  the  early  stage 
Tiave  disappeared;  sometimes,  indeed,  all  irritability  has  vanished. 
In  cases  that  are  seen  soon  after  the  onset  of  the  lesion  of  the  nerves, 
there  may  be  the  reaction  of  degeneration  in  the  muscles,  either  partiaJ 


LEPEOUS    NEUMTIS. 


195 


or  complete,  and  there  is  often  a  conspicuous  exaltation  of  the 
mechanical  irritability  of  the  nerve-trunks.  When  the  facial  nerves 
are  affected  this  may  be  well  seen,  and  may  resemble  that  met  with  in 
tetany.*  The  thickening  of  the  nerve  may  be  felt  when  that  which  is 
affected  is  accessible.  The  state  of  myotatic  irritabihty  varies  accord- 
ing to  the  distribution  of  the  neuritis ;  it  is  lost  at  places  where  the 
sensory  or  motor  fibres  are  involved,  but  those  supplying  the  front  of 
the  thigh  and  its  muscles  often  escape,  and  the  knee-jerk  is  preserved. 
Anchylosis  of  the  joints  may  occur,  as  in  other  forms  of  neuritis. 

The  "  mutilations "  of 
leprosy,  by  which  the  ends 
of  the  fingers  and  toes  are 
lost,  are  also  regarded  by 
some  as  a  consequence  of 
the  neuritis.  The  nume- 
rous other  symptoms  of 
the  disease  are  indepen- 
dent of  the  nerves  (except 
perhaps  the  pigmentation 
of  the  skin),  and  are  be- 
yond the  province  of  this 
book. 

The  neuritis  of  leprosy 
is  typically  adventitial 
(Fig.  63).  The  primary 
sheath  and  the  secondary- 
sheaths  of  the  fasciculi 
are  greatly  increased  in 
thickness,  and  consist  of 
nucleated  fibrous  tissue 
arranged  concentrically  (a, 
b).  From  the  sheath, 
tracts  extend  into  the  in- 
terior of  each  fasciculus 
(b),  isolating  the  groups 
of  nerve-fibres.  The  in- 
crease of  tissue  even  ex- 
tends between  the  fibres 
themselves  (c),  and  these 
undergo  slow  wasting ; 
many  of  the  fibres  in  the 

figxire  are  seen  to  be  distinctly  narrower  than  normal.  The  concentric 
growth  of  fibrous  tissue  may  even  invade  the  whole  area  of  the  fasci- 
culus, all  the  nerve-fibres  perishing  before  it  (d).  The  characteristic 
bacillus  of  leprosy  is  found  abundantly,  in  recent  cases,  in  the  new 
tissue  of  the  nerves.     Peculiar  cells  are  met  with,  infiltrated  with  the 


B~ 


Pia.  63. — Sections  of  nerves  from  a  case  of  anaes- 
thetic leprosy,  under  the  care  of  Dr.  Buzzard. 
A,  median  nerve  at  wrist  x  5;  B,  portion  of 
same  more  highly  magnified;  c,  part  of  a  less 
diseased  fasciculus  from  the  ulnar  nerve;  D,  a 
small  fasciculus  from  median  in  which  the  con- 
centric fibres  have  invaded  the  whole  area  of 
the  fasciculus. 


*  Schultze, 'Deut.  Arch,  f.kl  a.  Med.,' 1888,  Bd.  xliii. 


196  MULTIPLE    NEURITIS. 

organisms.  As  tlie  fibrous  tissue  develops  and  contracts,  tlie  bacilli 
seem  to  perish,  and  ultimately  can  no  longer  be  discovered. 

The  diagnosis  depends  on  the  occurrence  of  irregular  areas  of  anaes- 
thesia, generally  associated  with  irregular  patches  of  pigmentation 
and  pallor  in  the  skin,  and  often  with  muscular  atrophy,  in  a  person 
who  has  been  exposed  to  the  risk  of  infection,  generally  by  having 
lived  in  a  district  in  which  the  disease  is  endemic.  The  irregularity 
of  distribution  and  the  limitation  of  considerable  sensory  changes 
sufficiently  distinguish  it  from  other  forms  of  neuritis.  The  spinal 
affections  with  which  the  mixed  anaesthesia  and  wasting  are  most 
likely  to  be  confounded  are,  as  Schultze  has  pointed  out,  cases  of 
syringomyelia  in  which  the  distension  of  the  central  cavity  damages 
the  grey  matter,  and  may  cause  similar  symptoms  of  irregular  distri- 
bution— muscular  atrophy,  sensory  impairment,  trophic  disturbance.* 
But  these  are  limited  to  the  arms ;  and  if  the  legs  suffer  it  is  in  a 
different  way — as  simple  paraplegia  with  excess  of  myotatic  irritability 
that  may  go  on  to  spasm. 

The  prognosis  of  the  disease  in  the  simple  anaesthetic  form  is  grave 
only  when  the  sufferer  is  still  exposed  to  fresh  infection,  or  in  cases  of 
considerable  severity.  But  it  must  be  remembered  that,  just  as  the 
disease  may  develop  long  after  exposure  to  its  cause,  so  it  may  slowly 
increase  for  a  long  time  after  this  exposure  has  ceased. 

The  treatment  of  the  affection  is  beyond  the  scope  of  this  work ;  in 
so  far  as  the  nerve  symptoms  require  special  measures,  these  are  the 
same  as  in  ordinary  neuritis.  A  trial  may  be  made  of  the  stimula- 
tion of  the  muscles  by  whatever  form  of  electricity  they  will  respond 
to,  and  of  the  anaesthetic  areas  in  the  skin  by  the  wire  brush  and 
faradism. 

HERPETIC   NETJEITIS. 

Among  the  cutaneous  eruptions  occurring  in  acute  neuritis,  that 
which  characterises  "herpes  zoster"  has  been  mentioned.  This  affec- 
tion always  presents  a  correspondence  in  area  with  nerve  distribution ; 
a  definite  neuritis  has  been  found  in  the  rare  cases  which  have  afforded 
an  opportunity  for  pathological  investigation;  and  pain  with  other 
nerve  symptoms  is  common,  and  may  be  conspicuous.  Hence  herpes, 
although  usually  described  as  a  skin  disease,  is  really  the  manifestation 
of  a  form  of  neuritis  which  is  sufficiently  individual  in  course  and 
features  to  merit  a  separate  description.f 

Causes. — Our  knowledge  as  to  the  aetiology  of  the  neuritis  which 
underlies  the  most  common  form  of  herpes  is  still  very  incomplete. 
An  important  clue  is,  however,  afforded  by  the  fact,  first  pointed  out 
by  Mr.  Jonathan  Hutchinson,  that  the  disease  may  be  induced  by  the 

•  See  "  Mor\  an's  Disease,"  later. 

f  The  first  suggestion  as  to  the  neivous  origin  of  herpes  seemB  to  have  been  made 
by  Richard  Bright,  'Reports  of  Medical  Cases,'  1831,  vol.  ii,  part  1. 


HERPETIC    NEUEITIS.  197 

presence  of  arsenic  in  the  blood.  This  indicates  that  it  may  be  due 
to  a  chemical  toxic  agent  in  the  circulation,  and  suggests  that  as 
its  common  cause.  But  the  nature  of  the  agent  is  still  quite  un- 
known. The  careful  researches  of  Eendu  cast  doubt  upon  the  setio- 
logical  importance  once  attributed  to  gout,  although  a  causal  connection 
cau  occasionally  be  traced,  as  in  a  well-known  case  of  Trousseau's. 
Ebstein  *  records  the  case  of  two  brothers  who,  after  eating  mussels, 
developed  the  one  urticaria,  the  other  herpes.  The  affection  has  been 
traced  to  malaria  by  Winfield.f  We  know,  furthermore,  that  some  of 
the  many  varieties  of  rheumatic  toxins  may  act  upon  various  parts  of 
the  nervous  system,  in  a  manner  not  unlike  that  seen  in  poisoning  by 
arsenic,  so  far  as  concerns  the  characters  of  the  nerve  inflammation. 
Herpetic  neuritis  may  thus  probably  be  produced  by  toxins  introduced 
from  without,  or  manufactured  within  the  body  ;  it  may  even  assume 
an  epidemic  form.  We  do  not  know  why  it  should  usually  be  unilateral 
a.nd  show  a  predilection  for  certain  regions  ;  the  reason  is  probably  a 
local  one,  and  it  may  be  noted  that  herpes  has  been  seen  to  develop 
in  the  scar  of  a  burn,  and  to  reappear  in  those  left  by  a  first  attack 
forty-six  years  previously.  Neither  age  nor  sex  seems  to  have  much 
influence  in  relation  to  the  disease. 

Pathological  Anatomy. — The  indications  of  inflammation  have 
been  found  especially  in  the  ganglia  on  the  posterior  spinal  roots ;  they 
tend  to  spread  centrally  towards  the  cord,  rather  than  peripherally. 
The  Gasseiian  ganglion,  which  corresponds  to  that  of  a  posterior  root, 
is  similarly  affected  in  herpes  frontaHs.  Although  these  inflammatory 
changes  were  first  described  (by  von  Barensprung)  as  long  ago  as  in 
1861,  our  knowledge  as  to  their  exact  nature  is  still  meagre.  We  can 
state,  however,  that  they  affect  primarily  the  intercellular  material, 
and  that  they  are  ordinarily  very  severe,  frequently  inducing  hsemor- 
rhagic  exudation ;  the  swelling  and  injection  of  the  ganglia  and 
adjacent  nerve-trunks  are  usually  macroscopically  evident.  We  must 
distinguish  from  this  primary  form  those  in  which  the  eruption  is  only 
a  conspicuous  symptom  of  inflammation  communicated  to  the  nerve,  or 
clearly  secondary  to  some  other  cause.  Thus  Wagner  records  in  one 
case  purulent  pachymeningitis  and  swelling  of  the  spinal  gangha  of 
the  affected  area.  The  occurrence  of  bilateral  herpes  is  explained 
by  Kaposi  J  on  the  ground  of  primary  affection  of  the  spinal  cord, 
but  no  anatomical  proof  of  this  is  adduced,  and  it  seems  more 
reasonable  to  look  to  the  general  action  of  the  toxic  agent  in  the 
blood  as  the  cause.  As  to  the  nature  of  this  agent  we  are,  except 
in  the  case  of  the  special  poisons  already  mentioned,  absolutely 
ignorant.  This  point  has  already  been  discussed  in  connection  with 
neuritis. 

With  regard  to  distribution,  although  practically  any  sensory  nerve 

*  '  Vircliow's  Archiv,'  vol.  cxxxix. 
t  '  New  York  Med.  Journ.,'  1895. 
J  Euleiiberg's  '  Real  Eacyklopadie,'  art.  "  Herpeg." 


198  MULTIPLE    NEURITIS. 

may  stiffer,  the  main  incidence  is  upon  the  intercostals,  and  particu- 
larly those  of  the  lower  dorsal  region ;  the  fifth  nerve,  in  any  of 
its  branches,  but  particularly  the  first  and  second,  and  the  branches  of 
the  cervical  plexus,  giving  rise  to  the  occipito-cervical  form,  the  small 
occipital  and  great  auricular  nerves  being  most  affected.  The  lower 
part  of  the  leg  is  an  occasional  seat ;  the  arm  very  seldom  suffers. 
The  inflammatory  process  may  spread  to  the  adjacent  motor  nerves 
(see  later),  or  into  the  spinal  cord,  leading  in  one  case  to  death  from 
ascending  myelitis.*  The  herpes  itself  is  commonly  ascribed  to  im- 
plication of  trophic  nerves,  but  Ebstein  (loc.  cit.)  regards  it  as  due  to 
inflammatory  irritation  of  the  vaso-dilator  fibres. 

Symptoms. — The  description  of  the  herpetic  eruption  falls  properly 
within  the  province  of  dermatology.  Areas  of  congestion  appear 
with  oedema  passing  on  into  inflammation  with  effusion ;  the  epi- 
dermis is  raised,  and  pus  may  ultimately  form.  The  conspicuous 
commencement  in  certain  foci  is  probably  to  be  referred  to  the 
affection  of  terminal  nerve  divisions,  or  their  corresponding  ganglionic 
cells.  It  is  significant  of  a  general  toxic  cause  that  the  attack  may  be 
ushered  in  by  gastric  prodromata,  particularly  vomiting,  with  con- 
comitant fever.  Of  the  neuritic  symptoms  the  most  conspicuous  is 
pain,  which  will  be  more  fully  considered  under  the  head  of  neuralgia ; 
it  vsdll  here  suffice  to  note  the  distinctions  between  the  pre-  and  post- 
herpetic pains,  the  former  of  which  usually  abates  with  the  appearance 
of  the  rash,  while  the  latter  is  both  more  severe  and  of  longer  duration. 
Either  aneesthesia  or  hypersesthesia  of  the  region  served  by  the  affected 
nerve  frequently  follows  the  eruption ;  there  is  in  some  cases  per- 
sistent interference  with  conduction.  Motor  affections  are  also  not 
uncommon.  These  may  be  simply  immobihty  from  hypersesthesia  or 
true  paralysis  from  spreading  of  the  neuritis  to  motor  nerves.  In  the 
latter  case  there  is  some  muscular  atrophy  with  more  or  less  perfect 
reaction  of  degeneration.  Ebstein  has  collected  twenty  cases  of 
herpetic  palsy,  of  which  at  least  two  were  simply  hyperaesthetic 
immobility;  in  three  of  the  others  the  paralysis  preceded  the  erup- 
tion. The  facial  nerve  is  the  one  which  most  commonly  suffers,  and 
its  palsy  may  supervene  upon  herpes  of  the  trigeminal  or  occipito- 
cervical type.  In  a  case  recorded  by  Lesser,  frontal  herpes  was 
succeeded  by  paralysis  of  the  fourth  cranial  nerve,  the  inflammation 
having,  it  is  suggested,  spread  from  one  nerve  to  the  other  in  the 
ftavemous  sinus. 

As  regards  prognosis,  aU  that  need  be  said  is  that  the  severity  and 
persistence  of  the  neuritis  usually  vary  directly  with  the  age  of  the 
patient. 

The  question  of  the  treatment  of  herpes  does  not  fall  within  the 
scope  of  this  book ;  it  may,  however,  be  mentioned  that  the  pain  is 
frequently  alleviated  by  galvanism,  failing  which  cocaine  may  be  tried. 

*  Hardy,  '  Qaz.  des  Hopitaux,'  1876. 


PABT   Til. 
DISEASES   OF   THE   SPINAL  CORD. 


INTRODUCTION. 

ANATOMY  OF  TEE   SPINAL   CORD* 

The  spinal  cord,  it  will  be  remembered,  is  mucli  shorter  tLan  the 
spinal  canal,  reaching  only  to  the  second  lumbar  vertebra.  Hence  the 
nerve-roots  descend  to  their  foramina  of  exit.  The  lower  they  arise, 
the  longer  is  their  intra-spinal  course.  All  those  below  the  second 
lumbar  pair  leave  the  canal  below  the  lowest  portion  of  the  cord.  It 
is  customary  to  speak  of  the  portion  of  the  spinal  cord  from  which 
each  pair  of  nerves  arise  as  the  corresponding  "  segment"  of  the  cord. 
The  segments  are  longest  in  the  dorsal  region,  and  shortest  in  the 
Imnbar  enlargement.     They  are  also  called  "  metameres." 

The  only  parts  of  the  spinal  column  that  we  can  usually  feel  are 
the  vertebral  spines.  Mauy  of  these  are  not  on  a  level  with  the 
bodies  of  their  vertebrae.  It  is  important,  therefore,  to  know  the 
relation  of  the  spines  to  the  bodies  of  the  several  vertebrae,  and  of 
these  to  the  origin  of  the  nerves.  These  relations  are  shown  in  the 
accompanying  figure  (Fig.  64) . 

Of  the  Membranes,  the  pia  mater  closely  invests  the  surface,  and  is 
continuous  with  the  tracts  of  connective  tissue  that  pass  within  the 
substance  of  the  spinal  cord.  It  is  also  prolonged  along  the  nerve- 
roots  as  their  sheaths.  The  arachnoid  forms  a  much  less  close  invest- 
ment. The  dura  mater  is  not  in  contact  with  the  bones  as  it  is  in  the 
cranium,  but  a  layer  of  fat  and  a  plexus  of  large  veins  intei-vene 

*  The  following  outline  of  the  anatomy  of  the  spinal  cord  is  intended  merely  to 
place  before  the  reader  those  points  that  are  essential,  or  likely  to  become  so,  for 
understanding  the  functions  of  the  organ  and  the  symptoms  of  its  disease.  It  does 
not  profess  to  be  exhaustive  even  in  outline,  and  is  designedly  kept  as  free  as 
possible  from  whatever,  in  either  the  substance  or  terminology  of  recent  science, 
does  not  come  within  the  scope  of  those  objects.  Many  of  the  results  reached 
by  modern  investigators  are  mutually  incompatible,  and  where  a  choice  has  been 
necessary  the  observers  have  been  followed  who  possess  the  greatest  authority  and 
whose  conclusions  best  agree  with  facts  previously  ascertained. 


200 


SPINAL   OORD. 


a 


between  the  two.    It   thus  forms  a  loose    sheath    for    the    cord, 
and  variations  in  the  amount  of  blood  in  the  plexuses  outside  it 

permit  corresponding  (but  inverse)  varia- 
tions of   the  amount   of   cerebro-spinal 
fluid  within  it.     An   extension   of    the 
'  ^  dura  mater  passes  along  each  nerve-root, 
CI  0^^^^^         .   "     and  blends  with  its  sheath. 

Structure  of  the  Spinal  Cord. — 
The  general  form  of  the  cord,  the  enlarge- 
ments it  presents,  and  its  constitution  of 
white  and  grey  substance,  are  too  well 
known  to  need  description  here.  The 
wJiite  substance  surrounds  the  grey,  ex- 

^ '  (^..^'^SU-^-^-)- 1 D  cept  at  the  two    points  at  which  the 

2     posterior   horns    come    to    the    surface 

(Fig.   65).     It  consists   of    meduUated 

nerve-fibres,   chiefly   longitudinal.      The 

posterior  cornua    isolate    the    posterior 

^     columns  from  the  rest  of  the  white  sub- 

8  stance.     These  columns  are  separated  by 
7     a  "  posterior  median  septum  "  of  connec- 
tive tissue,  and  a  little  distance  from  this 
another  incomplete   septum,    "  posterior 

9  intermediate  septum,"  corresponding  to 
jg    a  depression  on  the  surface,  marks  off 

10    i^/r'^^JfTf  I  3-  portion  next  the  median  septum,  the 

^1 2'^=^'""    "         ^^    "  postero-median  column,"  or   "  column 

f^l^lStr'^"  '   "'"'"  ^^    °^  GroU,"  from  the  part  next  the  pos- 

"^  terior  horn,  "  postero-external  column," 


Fia.  64. — Diagram  (framed  from  an  original  in- 
— .  -    3  vestigation)  showing  the  relation  of  the  verte- 

bral spines  to  their  bodies  and  to  the  origin  of 
g  the  several  nerve-roots.     It  will  be  seen  that 

.-'iijllj  m the  ends  of  the  vertebral  spines  are  opposite 

C-^/lll  \  ^  I  *^®   middle  of  their  own  bodies  only  in  the 

—    ^  lumbar  region;  they  correspond  to  the  lower 

edge  of  their  own  bodies  in  the  cervical  and 

■"" °  the  first  two  dorsal  vertebrsfi,  and  to  the  upper 

part  of  the  body  below  in  the  rest  of  the  dorsal 
j  s      region.     Each  cervical  spine  is  nearly  opposite 
the  lower  roots  of  the  nerve  below;  the  vertebra 
2  prominens  is  opposite  the  first  dorsal  roots,  nnd 

from  the  3rd  to  the  10th   dorsal    the   spines 
correspond  to  the  second  root  below;  the  11th 

4  spine  corresponds  to  the  1st  and  2nd  lumbal 

nerves,  the  12th  to  the  3rd,  4tl),  and  5th ;  the 

■"' ■    ^  1st   lumbar  to  the   1st,  2nd,  and  3rd  sacral 

0°       nerves,  while  the  tip  of  the  cord  is  opposite 

the  upper  part  of  the  2nd  lumbar. 

or  "column  of    Burdach,"   the    part    of    which    next    the    cornu 
is  caUed  the   "posterior  root-zone,"   because  many    fibres    of   the 


STBUOTUKE. 


201 


posterior  root  pass  through  it.     The  rest  of  the  white  substance  is 
diyided,  in  the  middle  line  in  front,  by  the  anterior  median  fissm-e, 


Fiff.  65. — Diaeram  of  a  section  of  the  spinal  cord  in  the  cervinal  region.  A..  C, 
anterior  commissure;  P.  C,  posterior  commissure;  I.  g.  s.,  intermediate  grey  sub- 
stance; p.  Cor.,  posterior  cornu;  c.  c.  p.,  caput  eorna  posterioris;  L.  L.  L.,  lateral 
limitinsr  layer;  a.-l.  a.  t.,  antero-lateral  ascending  tract,  which  extends  along  the 
periphery  of  the  cord. 


down  which  the  pia  mater  a.nd  blood-vessels  pass,  and  at  the  bottom 
of  which  is  the  anterior  or  white  commissure.  Between  the  anterior 
median  fissure  and  the  posterior  cornu  the  white  substance  is  con- 
tinuous and  undivided,  extending  round  the  front  and  side  of  the 
cord.  It  is  artificially  divided  into  an  anterior  and  a  lateral  column, 
the  line  of  division  being  the  outermost  of  the  anterior  nerve-roots, 
which  pass  through  the  front  of  the  cord ;  but  there  is  no  corre- 
sponding distinction  of  structure,  and  hence  it  is  often  termed  the 
**  antero-lateral  column." 

The  white  substance  varies  in  amount  in  different  parts  of  the 
cord,  but,  as  a  whole,  lessens  gradually  from  above  downwards  (see 
Fig.  66).  It  is  everywhere  composed  of  niedullated  nerve-fi.bres, 
which,  however,  possess  no  neurilemma-sheath.  In  carmine- stained 
sections  the  axis-cylinder  is  seen  within  each  fibre,  not  always  in  the 
centre,  and  around  this  are  concentric  cloudy  lines  due  to  the  irregular 
refraction  of  the  white  substance. 

Between  the  fibres  is  a  peculiar  substance,  the  "  neuroglia,*'  or 
nerve-cement.  It  appears  to  consist  of  nucleated  branched  cells 
("spider-cells"),  whose  long,  delicate  processes  form  the  fibres  of 
the  neuroglia.  The  cells,  stellate  on  section,  are  found  in  the  larger 
interspaces  between  the  nerve-fibres.  The  fibres  of  the  neuroglia 
branch  and  interlace,  forming  a  very  fijie  network;   some  of  them 


202 


SPINAL    COED. 


are   arranged   radially;    starting  centrally  from   the   outer  ends  of 
the  ciliated  epithelial  cells  which  line  the  cerebro- spinal  canal,  they 

diverge,  branching  as  they  go 
towards  the  surface  of  the  cord. 
This  neuroglia  is  developed  from 
the  same  (epithelial)  embryo- 
logical  elements  as  the  nerve- 
structures — an  important  fact, 
because  it  enables  us  to  under- 
stand the  fact  that,  in  defective 
development  of  the  nerve- struc- 
tures, the  neuroglial  tissue  is  ex- 
cessive in  amount.  But  its  differ- 
entiation in  development  involves 
also  its  chemical  nature,  since  it 
contains  far  more  of  a  material 
like  that  of  hair  or  horn,  and 
hence  called  "  neurokeratin." 

The  nerve-fibres,  thus  con- 
nected, lie  in  a  coarser  network, 
formed  by  branching  processes 
of  septa  that  proceed  inwards 
from  the  pia  mater.  In  these 
septa  blood-vessels  run,  and  some 
are  occupied  by  nerve-fibres  that 
have  a  horizontal  course.  The 
septa  consist  of  fine  fibres  of 
ordinary  connective  tissue  from 
the  pia  mater,  and  of  neuroglial 
material,  which  forms  also  a  layer 
between  the  membrane  and  the 
nerve-fibres  in  the  intervals  be- 
tween the  septa.  This  layer  varies 
considerably  in  thickness.  Many 
of  the  septa  pass  through  the 
white  substance  to  join  projec- 
tions from  the  grey  substance, 
which  cause  the  irregularity  of 
the  outline  of  the  latter.  These 
processes,  outside  the  neck  of  the 
posterior  cornu,  are  broad,  and  join 
each  other  so  as  to  form  a  sort  of 
network,  the  meshes  of  which  en- 
close columns  of  nerve-fibres.  This 
is  called  the  "  lateral  reticular  f  or- 
Fia.  66.— Diagram  showing  the  relative  mation  "  (Fig.  65,  right  side  ;  also 
size  and  shape  of  the  cord  and  orey       „^       „      on      rm  ^    j.  i?  j.i 

matter  at  diaWit  levels.  66,  c  2—8).    The  substance  of  the^ 


STRUCTURE. 


203 


horn  behind  the  caput  is  sometimes  broken  up  in  the  same  way— 
the  "  posterior  reticular  formation." 

The  grey  substance  varies  in  shape  and  size  in  different  parts  of  the 
cord,  being  largest  in  the  cervical  and  lumbar  swellings,  and  corre- 
sponding to  the  number  and  size  of  the  nerve-roots  given  off.  The 
variations  in  shape  and  size  are  indicated  in  the  accompanying  figure 
(JoQ),  and  will  be  readily  understood  by  an  examination  of  this.  The 
division  into  anterior  and  posterior  horns  or  cornua  is  familiar.  The 
part  on  each  side  that  intervenes  between  the  two  horns  may  be 
conveniently  termed  the  "  intermediate  grey  substance  '•'  (I.  g.  s.  in 
Fig.  65).  In  the  dorsal  region  a  projection  from  this  extends  into 
the  lateral  column  (Fig.  Q&,  d  2,  5,  and  8),  and  has  been  termed  the 
"lateral  horn").  It  is  customary  to  divide  the  grey  matter  into 
two  varieties,  "spongy"  and  "gelatinous."  The  latter  forms  a 
cap  on  the  posterior  horn,  and  a  layer  immediately  around  the  central 
canal,  and  is  named  on  account  of  its  naked-eye  aspect.  It  consists 
largely  of  neuroglia,  traversed,  however,  by  nerve- fibres,  and  with 
numerous  nerve-cells  scattered  through  it.  The  spongy  substance, 
which  forms  the  rest  of  the  two  cornua,  consists  mainly  of  an  exces- 
sively fine  f  elty  network  of  very  narrow  meduUated  nerve-fibres,  naked 
axis-cylinders,  and  fine  nerve-fibrillse  which  arise,  in  part  at  least, 
from  the  dendrites  of  the  nerve-cells,  and  in  part  by  a  division  of 
the  axis-cylinders  of  the  nerve-fibres.  These  structures  are  em- 
bedded in  a  supporting  neuroglia.  Through  it  course  many  larger 
medullated  nerve-fibres,  passing  to  or  from  the  white  columns  and 
nerve-roots.  In  it  also  lie  many  nerve-cells  of  various  sizes.  Those 
in  the  anterior  cornu  are,  for  the  most  part,  large  "  ganglion-cells  ;  " 
each  contains  a  large  nucleus,  and  usually  a  mass  of  pigment,  and 


Fig.  67. 


Fia.  68. 


Fia.  69. 


Fig-.  67. — A  nerve-cell  from  tlie  anterior  cornu  of  the 
spinal  cord  of  man:  a,  uubranched  process  becoming 
the  axis-cylinder  of  a  nerve-fibre;  b,  pigment  mass. 
(After  Gerlach.) 

Fig-.  68. — Nerve-cells  of  the  anterior  cornu  sending  a  pro- 
cess into  tl  e  anterior  root.     (After  Henle.) 

Fig.  69. — A  fine  nerve-fihre  dividiii'i'  into  two  parts,  each 
of   whiih  joins  the  plexus  of   fibrillse  formed  by  the 
branching  processes  of   a  nerve-cell.     From  the  spinal  cord  of    au  ox. 
Gerlach.) 


(After 


204  SPINAL    CORD. 

sends  out  several  processes.  One  process,  the  axon,  is  continuous 
with  the  axis-cylinder  of  a  nerve-fibre  (Fig.  &?),  and  can  be  sometimes 
traced  into  a  fibre  of  an  anterior  root  (Fig.  68).  The  other  processes, 
or  dendrons,  after  a  longer  or  shorter  course  divide  and  subdivide, 
their  ramifications  ending  in  minute  knobs  in  the  sponge-like  matrix 
of  the  grey  substance.  In  this  they  are  probably  brought  into  close 
contiguity  with  the  terminal  ramifications  of  other  neurons  (Fig.  69) . 
These  cells  lie  in  groups,  between  and  through  which  course  tracts  of 
fibres,  chiefly  from  the  anterior  roots.  Other  fibres  come  from  the 
anterior  commissure,  white  columns,  and  posterior  horn.  The  axons 
of  these  cells  are  certainly  motor  in  nature,  and  become  the  motor 
fibres  of  the  spinal  nerves.  Similar  cells  are  also  found  in  varying 
numbers  at  the  anterior  periphery  of  the  cord,  and  even  among  the 
fibres  of  the  already  descending  anterior  roots.*  Birge,  in  a  careful 
investigation,  found  that  in  each  segment  of  the  spinal  cord  of  the 
frog  the  number  of  nerve-cells  in  the  anterior  cornu  nearly  corresponds 
to  the  number  of  fibres  of  the  anterior  roots  arising  from  that  segment. 
Each  ceU  is  traversed  by  many  nerve-fibrillse,  such  as  are  discerned  in 
a  cell-process  or  in  the  axis-cylinder  of  a  nerve-fibre.  These  cross  and 
intermingle  in  the  middle  of  the  cell,  but  pass  along  the  margin  from 
one  process  to  another.  By  this  arrangement  impulses  entering  the 
cell  by  one  process  may  leave  it  by  many.f 

The  arrangement  of  the  nerve-cells  of  the  anterior  cornu  is  of  some 
practical  importance,  on  account  of  the  frequency  with  which  this  part 
is  the  seat  of  limited  lesions.  They  form,  as  already  stated,  certain 
groups,  but  these  groups  vary  in  different  regions  of  the  cord,  partly 
under  the  influence  of  the  shape  of  the  horn,  partly  irrespective  of 
this ;  and  they  even  vary  in  parts  of  the  cord  that  are  near  together. 

They  are  influenced  by  the  course  of  the  tracts  of  fibres  of  the  ante- 
rior roots,  which  may  pa,ss  through  a  group  and  break  it  up  into  smaller 
groups,  although  in  a  neighbouring  section  it  is  undivided  (see  Fig.  70, 
the  cervical  groups).  Hence  very  different  descriptions  have  been 
given  of  these  groups,  and  the  process  of  distinction  has  sometimes 
been  carried  too  far.  The  groups  that  can  most  readily  be  recognised 
are  the  following : — In  the  inner  anterior  angle  of  the  cornu  is  a  small 
group,  the  inner  or  median  group.  This  is  one  of  the  smallest,  and  is 
absent  in  some  parts  of  the  cord,  especially  in  the  lumbar  region.  A 
much  larger  group  lies  near  the   anterior  edge  of  the  horn,  in  the 

*  Hoche,  'Neurol.  Cenfcralbl.,'  1891. 

t  Kronthal,  *  Neur.  Centralbl.,'  1890,  p.  40.  Impulses  may  thus  be  difFused,  even 
if  there  is  no  junction  of  fibrillsB  or  passage  of  impulses  from  one  fibril  to  another. 
This  arrangement  provides  a  mechanism  by  which  the  nerve-cells  maj'  conduct  and 
diffuse  impulses,  but  such  a  mechanism  does  not  exclude  (as  has  been  assumed)  the 
reinforcement  or  origination  of  such  impulses,  and  their  discovery  leaves  untouched 
the  facts  which  demonstrate  the  trophic  influence  of  the  cells,  and  the  manner  in 
which  they  arrest  secondary  degeneration.  These  functions  are  indicated  by  facts 
of  a  totally  different  nature.  Kronthal's  observations  are  of  great  importance,  and 
appear  trustworthy. 


STRTJCTDHE. 


205 


middle,  or  a  little  to  the  outer  side  of  the  middle,  the  anterior  group. 
External  to  this,  in  the  outer  extremity  of  the  front  part  of  the  horn, 
is  another  group,  the  antero-lateral  group.  Ihese  two  groups  are 
often  blended,  so  that  the  anterior  group  cannot  be  separately  distin- 
guished, as  in  the  right-hand  mid-cervical  figure.  A  fourth  group, 
usually  the  largest,  Ues  in  the  outermost  part  of  the  horn,  behind  its 
front,  usually  in  the  posterior  outer  angle ;  it  may  extend  inwards, 
halfway  across  the  horn.  It  is  called  the  external  or  postero-lateral 
grou^f  (P.-L.,  Fig.  70).    These  three  are  the  most  important  groups. 


Upper  Dorsal 


Mid-Lumbar. 


FlS.  70. — Diagrams  of  the  groups  of  nerve-cells  in  the  anterior  cornu.  Groups  : — 
1.,  inner  or  medial ;  A.,  anterior ;  a.-l.,  antero-lateral ;  p.-l.,  postero-lateral ;  I.  L.  p., 
intermediate  lateral  process ;  p.  T.  c,  posterior  vesicular  column  or  tract.  The  two 
mid-cervical  sections  are  only  a  few  millimetres  apart,  and  show  how  the  anterior 
group,  separate  in  the  one,  may  be  blended  with  the  antero-lateral  group  in  a 
neighbouring  part  of  the  cord. 


There  is  in  some  parts  also  a  central  group,  occupying  nearly  the  centre 
of  the  horn.  In  the  small  cornu  of  the  dorsal  region  often  no  weli- 
defined  groups  can  be  made  out,  but  when  any  can  be  recognised,  they 
are  generally  the  anterior  and  external. 

Similar  cells,  usually  smaller  in  size  and  isolated,  are  scattered 
through  the  intermediate  grey  matter,  and  a  group  of  cells  occupies  a 
projection  outwards  into  the  lateral  column  in  the  lower  cervical  and 
upper  dorsal  regions.  It  was  termed  by  Lockhart  Clarke  the  inter- 
media-lateral  tract,  but  is  better  designated  the  intermediate  process 
(or  lateral  horn),  and  the  cells  the  intermediate  group  (i.  l.  p.,  Fig.  70). 


206  SPINAL   CORD. 

Nerve- cells  also  lie  in  the  grey  matter  of  the  reticular  formations, 
lateral  and  posterior,  and  are  sometimes  described  as  special  groups. 
Many  isolated  colls,  mediiim  and  small  in  size,  also  lie  in  the  posterior 
horn.  These  are  probably  connected  with  some  of  the  fibres  of  the 
posterior  roots.     They  extend  back  into  the  caput  comu  posterioris.* 

Throughout  the  dorsal  and  in  the  upper  lumbar  cord  a  group  of 
nerve-cells  lies  in  the  inner  part  of  the  neck  of  the  posterior  horn,  the 
posterior  vesicular  column  of  Lockhart  Clarke  (sometimes  called 
"Clarke's  column")  (p.  v.  c.  Fig.  70;  see  also  Fig.  80).  Most  of 
these  cells  are  fusiform  in  shape,  but  are  placed  vertically,  so  that  they 
appear  round  or  oval  in  transverse  section,  and  their  processes  are  not 
seen.  Some,  however,  present  processes  that  run  forwards  or  back- 
wards. Nerve-fibres  pass  into  and  by  it,  which  we  shall  consider  sub- 
sequently. Although  this  tract  is  chiefly  developed  in  the  lower  dorsal 
and  upper  lumbar  cord,  a  few  nerve-cells  of  similar  character  are  met 
with  in  the  same  position  in  other  parts,  and  are  occasionally  suffi- 
ciently numerous  to  form  a  small  group. 

The  "  gelatinous "  grey  matter,  grey  substance  of  Rolando,  which 
forms  a  cap  on  the  posterior  horn,  caput  comu  posterioris  (c.  c.  p.. 
Pig.  65),  differs  considerably  from  the  spongy  substance.  It  has 
been  described  as  made  up  of  granules,  but,  according  to  later 
researches,  consists  of  a  peculiar  translucent  material  forming  an 
excessively  fine  network,  which,  like  the  'rest  of  the  neuroglia,  behaves 
to  reagents  like  horny  material.  In  the  embryo  it  is  contiauous  with 
that  which  surrounds  the  central  canal,t  but  from  this  it  becomes 
separated  in  the  course  of  development.  Its  basis  substance  is  thus 
of  neuroghal  natiire.  Numerous  nerve-fibres  pass  through  it  from 
the  posterior  roots,  and  these,  coursing  forwards,  seem  to  divide 
it  into  columns,  as  it  is  viewed  in  transverse  section.  A  few  small 
ganglion-cells  lie  in  this  gelatinous  substance,  chiefly  on  the  inner 
side ;  it  also  contains  many  minute  cells,  some  of  which  are 
nerve-cells,  while  others  belong  to  the  neuroglial  tissue.  From 
the  nerve-cells  axons  have  been  traced  into  the  posterior  column  and 
horn,  the  posterior  root-zone  and  the  lateral  column.J  Bundles  of 
vertical  fibres  are  also  seen  in  transverse  section,  and  these  are  often 
numerous  just  above  the  caput,  especially  on  the  inner  side  of  the 
horn.  Most  of  these  are  fibres  of  the  posterior  nerve-roots  that 
have  a  vertical  course  for  a  short  distance.  In  Fig.  80  the  axis- 
cylinders  of  these  fibres  are  seen  to  be  changing  from  the  horizontal 
to  the  vertical  direction,  and  their  relation  to  the  posterior  root- 
fibres  can  be  distinctly  traced.     The  hinder  part  of  the  gelatinous 

•  It  is  generally  believed  tbat  their  branching  procesBes  enter  into  the  plexus  of 
fibrillse  in  the  grey  matter,  and  thus  bring  these  cells  into  relation  with  those  of  the 
anterior  cornua,  and  also  with  fibres  that  cross  by  the  posterior  commissure  and 
form  the  ascending  sensory  path  to  the  brain. 

t  Cf.  Corning,  '  Arch.  f.  mikios.  Anat.,'  xxxi,  1888. 

X  Starr,  '  Atlas  of  Nerve-cells,'  p.  28  ;  Leuhossek,  •  Der  feinere  Baa  des  Nerven. 
systems,'  pp.  120  et  seq. 


STRUCTURE.  207 

■ubstance  is  less  dense  in  structure  than  tlie  rest,  and  is  sometimes 
distinguislied  from  it  as  the  "  spongy  zone."*  The  gelatinous  sub- 
stance contains  many  vessels,  running  for  the  most  part  vertically. 

White  Substance. — We  may  now  examine,  in  greater  detail,  the  con- 
stitution of  the  white  columns  of  the  cord,  and  the  probable  course  and 
function  of  the  fibres  they  contain.  Their  analysis  has  been  greatly 
aided  by  three  facts, — continuity  of  degeneration,  difference  in  time  of 
development,  and  the  variation  in  the  size  of  the  fibres  of  the  different 
tracts.  1.  It  was  discovered  by  Tiirck  that  in  certain  tracts  in  the 
white  substance  the  nerve-fibres  undergo  secondary  degeneration  when 
separated  from  their  cells  of  origin,  a  degeneration  analogous  to  that 
which,  as  we  have  already  seen,  occurs  in  the  nerve-fibres  outside  the 
cord.  This  mode  of  investigation  has  of  late  acquired  a  fresh 
impetus  from  the  new  means  of  staining  degenerated  nerve-fibres 
devised  by  Marchi.  2.  In  the  developing  cord  the  fibres  of  different 
tracts  acquire  their  white  substance  at  different  periods,  and  the  study 
of  these  differences  (first  by  Flechsig  and  later  by  Bechterew  and 
others)  has  not  only  confirmed,  but  also  extended,  the  differential 
indications  afforded  by  disease.  It  enables  us  to  make  a  distiaction 
between  fibres  that  do  not  degenerate  separately,  or  not  through  a 
sufficiently  long  tract  to  enable  us  to  distinguish  them.  The  groups 
into  which  this  method  enables  us  to  divide  the  fibres,  and  the  order 
in  which  the  meduUation  takes  place,  are  as  follows : 

(1)  Anterior  and  posterior  root-fibres. 

(2)  -N  r  inner  anterior  column. 

(3)  >  Ground-fibres  of  the  <  postero-external  column. 

(4)  J  (.  outer  anterior  and  lateral  column. 
(6)  Lateral  limiting  layer  and  antero-lateral  ascending  tract. 

(6)  Postero-median  column. 

(7)  Direct  cerebellar  tract. 

(8)  Lateral  and  anterior  pyramidal  tracts  (which  in  man  are  first 
medullated  at  the  time  of  birth)  .f  It  may  be  stated  as  a  general 
rule  that  the  shorter  the  course  of  a  tract  within  the  central  nervous 
system,  the  earlier  do  its  fibres  acquire  their  medullary  sheaths. 

3.  The  size  of  the  nerve-fibres  varies  in  different  tracts,  but  the 
full  significance  of  the  variations  has  still  to  be  ascertained.  Even  in 
the  same  tract  of  the  cord  considerable  variations  are  met  with,  which 
probably  correspond  with  differences  in  function.  "When  a  tract  dege- 
nerates, it  is  common  for  the  degeneration  to  be  incomplete ;  fibres 
remain  intact  among  those  that  perish :  this  indicates  a  difference  in 
function,  and  with  it  the  difference  in  size  usually  coincides.  It  is  also 
possible  that  some  of  the  small  fibres  seen  among  larger  ones  may  be 
due  to  the  division  of  the  latter  (Sherrington)  ;  but  such  division 

•  So  that  in  some  descriptions  the  posterior  horn  is  divided  into  (1)  spnngy  sub- 
stance of  the  horn  itself,  (2)  gelatinous  zone  of  the  "  caput,"  and  (3)  spougy  zone 
at  the  ti])  of  the  horn.     See  Lissauer, '  Ai*ch.  f.  Psych.,'  1886. 

t  See  Obersteiner,  loc.  cit.,  p.  192. 


208 


SPINAL   COED. 


has  not  been  actually  demonstrated,  and  is  probably  not  frequent. 
The  variation  in  size  is  from  1  to  25  n  (tswo  to  toVo  inch).  The 
variations  are  especially  great  in  the  fibres  of  the  pyramidal  tract, 
and  it  is  in  these  that  division  has  been  thought  to  occur.  Those  of 
the  direct  cerebellar  tract  are  uniformly  large,  while  most  of  those 
that  constitute  the  postero-median  column  are  small. 

It  will  facilitate  the  detailed  study  of  the  tracts  if  we  consider 
first  those  that  undergo  secondary  degeneration  for  a  considerable 
distance,  as  our  knowledge  regarding  these  is  the  most  complete. 


o.a 


L.4 


Fig.  71.— Diagram  of  the  elements  of  the  white  substance  at  various  levels 
of  the  spinal  cord.  (Modified  from  Flechsig.)  The  pyramidal  tracts  are 
shaded  by  short  horizontal  lines ;  the  direct  cerebellar  tract  by  dots;  the 
posterior  median  column  by  dots,  as  it  degenerates  in  disease  of  the  lowest 
part  of  the  cord  or  of  the  nerves  of  the  cauda  equina  %  the  small  circles 
in  the  forepart  of  this  column  indicate  the  area  which  degenerates  in 
disease  of  the  cervical  enlargement.  The  antero-lateral  ascending  tract 
ia  shown  by  crosses.  The  anterior  ground-fibres,  lateral  limiting  layer, 
and  postero-external  column  are  left  white.  Fig.  66  should  be  compared 
with  this. 


STEUCTUEB.  209 

These  degenerations  are  both  ascending  and  descendiag.  Only  one 
important  set  of  fibres  degenerates  downwards  through  a  considerable 
extent — those  that  continue  through  the  cord  the  anterior  pyramids 
of  the  medulla.  They  commence  still  higher,  in  the  motor  region  of 
the  cortex  of  the  cerebral  hemisphere,  passing  thence  by  the  "  internal 
capsule  "  between  the  two  parts  of  the  corpus  striatum,  &c.,  to  the 
cms  and  pons;  they  are  the  chief,  perhaps  the  sole  conductors  of 
voltmtary  impulses.  They  are  termed  the  pyramidal  tracts  because 
they  constitute  the  anterior  pyramids  of  the  medulla.  They  are  shaded 
by  short  horizontal  lines  in  Fig  71 ;  compare  also  Fig.  65. 

At  the  decussation  of  the  pyramids  about  three  quarters  of  the 
fibres  usually  cross  to  the  other  side.  These  pass  down  the  cord  in 
the  lateral  column,  and  constitute  the  lateral  (or  crossed)  pyramidal 
tract.  Those  which  do  not  decussate,  pass  down  their  own  side  of  the 
cord,  in  the  inner  part  of  the  anterior  column,  at  the  side  of  the 
anterior  median  fissure,  and  constitute  the  anterior  (or  direct)  pyra- 
midal tract.  Flechsig  has  found  that  the  decussation  is  subject  to 
many  variations.  In  the  majority  of  cases  the  decussating  fibres  are 
between  70  and  80  per  cent,  of  the  whole.  Sometimes  they  con- 
stitute a  still  larger  proportion,  and  in  one  case  (of  60  examined)  all 
the  fibres  crossed.  Sometimes  fewer  fibres  decussate,  only  one  half 
or  even  less  than  half ;  in  one  case  35  per  cent.,  in  another  only  10. 
No  case  has  yet  been  met  with  in  which  no  fibres  crossed.*  A  con- 
siderable number  of  fibres  decussate  above  the  medulla,  terminating 
around  the  cells  which  give  origin  to  the  motor  cranial  nerves. 

The  lateral  pyramidal  tract  occupies  the  posterior  half  of  the  lateral 
column,  outside  the  posterior  cornu.  It  extends  down  to  the  end  of  the 
cord,  even  when  it  is  originally  small.  Hence  those  fibres  that  do  not 
cross  at  the  medulla  must  do  so  lower  down  in  the  cord.  Through  the 
greater  part  of  the  cervical  and  dorsal  regions  this  lateral  tract  is 
separated  from  the  surface  by  a  narrow  layer  of  fibres,  the  direct  cere- 
bellar tract.  In  the  upper  part  of  the  cervical  region  (third  cervical 
segment)  this  tract  lies  farther  forwards,  so  that  the  pyramidal  tract 
comes  up  to  the  surface  close  to  the  posterior  cornu  (Fig.  71,  c.  8) ; 
and  here,  if  the  tract  is  small,  there  may  be  a  depression  on  the  sur- 
face. In  the  lower  part  of  the  dorsal  cord  there  is  a  similar  move- 
ment forwards  of  the  cerebellar  tract,  so  that  the  pyramidal  tract 
again  comes  in  contact  with  the  surface  posteriorly  (Fig.  71,  d.  12), 
and,  as  the  cerebellar  tract  ceases  at  the  first  lumbar  nerve,  the  pyra- 
midal tract  extends  up  to  the  surface  throughout  the  lumbar  enlarge- 
ment (Fig.  71,  L.  4).  The  inner  side  of  tlie  tract  is  in  contact  with  the 
hinder  part  of  the  posterior  cornu,  near  the  surface,  throughout  the 

•  In  the  'Neur.  Cent.,*  1898,  p.  202,  Dr.  Philip  Zenner  describes  a  case  o£ 
tumour  of  the  left  side  of  the  brain  in  which  the  paralysis  was  on  the  left  side  of 
the  body.  The  post-mortem  examination  showed  th;it  tliere  was  "absence  of  tlie 
pyramidid  decussation."  It  ia  not  stated  whether  Marchi's  method  was  used  in  the 
examination. 

VOL.   I.  14 


210 


SPINAL    COED. 


entire  lengtli  of  tlie  cord,  but  further  forwards  it  is  separated  from  the 
neck  of  the  cornu  and  intermediate  grey  substance  by  a  layer  of  nerve- 
fibres,  termed  by  Flechsig  the  lateral  limiting  layer  (l.  l.  l..  Fig.  65). 
Among  the  fibres  of  the  pyramidal  tract,  in  the  dorsal  and  cervical 
regions,  degeneration  reveals  a  few  other  fibres  that  belong  chiefly 
to  the  cerebellar  tract.  The  lateral  pyramidal  tract  diminishes  in 
size  from  above  downwards  as  its  fibres  end  by  entering  the  grey 
matter ;  those  that  extend  lowest  (for  the  leg)  occupy  the  posterior 
part  of  the  tract  in  the  cervical  region.  The  fibres  curve  inwards 
along  the  septa  that  extend  from  the  grey  matter  into  the  lateral 
column,  and  they  enter  the  grey  matter  between  the  anterior  and 
posterior  cornua.  They  pass  inwards  and  forwards  in  the  anterior 
cornu,  and  are  then  lost  in  the '  complicated  structure  of  the  grey 
matter.  Their  probable  termination  will  be  considered  presently. 
The  anterior  or  direct  pyramidal  tract  (called  also  the  "  column  of 

Tiirck  ")  descends  the  cord  in  the  inner 
part  of  the  anterior  column,  adjacent 
to  the  anterior  median  fissure.  Its 
exact  form  and  size  vary :  usually  it 
bounds  the  fissure  on  each  side ;  some- 
times it  forms  only  a  small  tract  at 
the  posterior  part  of  the  fissure  ;  when 
large,  it  not  only  reaches  forward  to 
the  front  of  the  cord,  but  may  cause  a 
pr-^minence  on  the  surface  bounded  by 
a  distinct  sulcus.  Its  extent  down  the 
cord  probably  varies  according  to  its 
size.  It  steadily  lessens  in  area,  and, 
if  originally  small,  may  not  extend 
beyond  the  middle  of  the  cervical  en- 
largement ;  if  originally  large,  it  may 
be  traced  as  far  as  the  lumbar  enlarge- 
ment and  even  into  the  cord  at  the 
cervical,  and  shows  the  ascending  level  of  the  fourth  pair  of  sacral  nerve- 
degeneration  of  the  post..median  ^^  .j,-  ^^y  -^  bearing  the 
column  spreading  out  against  the    •'""  "       v-^   »     _     y  '  & 

commissure.  The  other  figure  is  USual  proportion  to  the  lateral  tract, 
from  the  lower  dorsal,  and  shows  j^  ceases  about  the  mid-dorsal  region, 
degeneration    ot    the    pyramidal    ^^     ,       „  ,,       „,  ,,  i     j^-. 

tracts,  anterior  and  lateral.  Most  of  the  fibres  pass  through  the 

anterior  commissure  to  the  opposite 
side,  either  entering  at  once  the  grey  matter,  or  passing  first  to  the 
lateral  tract.f  The  latter  is  probably  the  course  of  most  of  the  fibres 
when  the  pyramidal  decussation  is  small.  Whether  any  fibres  end 
in  the  grey  matter  on  the  same  side  of  the  cord  we  do  not  know. 

Disease  of  the  brain  destroying  the  motor  cortex,  or  the  fibres  which 

*  Dejerine  and  Thomas,  '  Compt.  Rend.,'  1896,  p.  157.  Russell  traces  it  to  the 
5th  segment. 

t  Although  this  is  probable  there  is  no  definite  evidence  of  it  except  in  the  sacral 
yegiou  (Russell, '  Brain,'  1898). 


Fio:  72 — Ascending  and  descend- 
ing degenerations  from  myelitis 
of  the  mid-dorsal  region ;  the 
upper  section  is  from  the  lower 


STETJCTUEE. 


21] 


descend  from  it  to  tlie  pyramids,  causes  desc^nrling  degeneration. 
■  usually  limited  (at  least  in  conspicu- 
ous degree)  to  tlie  related  pyra- 
midal tracts, — anterior  on  tlie  same 
side,  lateral  on  the  opposite  side 
(Fig.  73) .  But  in  many  cases  sligM 
degeneration  lias  been  also  found 
in  the  lateral  tract  on  the  same  side, 
extending  into  the  lumbar  region. 
Hence  it  is  probable  that  some 
fibres  of  each  anterior  pyramid 
find  their  way  to  the  lateral  tract  on 
the  same  side,  and  descend  to  the 
corresponding  limbs,  especially  to 
the  leg.  This  degeneration  of  the 
lateral  tract  on  the  same  side  as 
the  lesion  is  sometimes  very  marked 
just  below  the  decussation  of  the 
pyramids,  and  is  therefore  not  due 
to  any  lower  re-decussation  in  the 
cord.*  Muratoff  has  shown,  by 
extirpation  of  the  cortical  areas  for 
the  extremities,  that  a  bundle  from 
the  bulbar  pyramid  descends  into 
the  lateral  pyramidal  tract  of  the 
same  side.  This  has  been  con- 
firmed by  Eiothmann  and  also  by 
Melius  and  by  Sherrington,  who  for- 
merly held  a  different  view.f  That 
there  is  such  a  physiological  relation 
is  clearly  indicated  by  clinical  facts. 
We  shall  afterwards  see  that  there 
is  abundant  evidence  that  each 
hemisphere  of  the  brain  is  con- 
nected with  both  legs,  although 
chiefly  with  that  of  the  opposite 
side.  There  is  also  a  similar  and 
even  more  equal  connection  with  the 
trunk  muscles,  and  a  slighter  con- 
nection with  certain  muscles  of  the 
arm.  Such  muscles  invariably  re- 
gain some  power  on  the  paralysed 
side,  even  with  a  complete  inter- 


PlO.  73. — Descending  degeneration  of 
the  pyramidal  tracts  in  a  case  of 
hemiplegia  from  disease  of  the  right 
cerebral  hemispiiere.  (Prom  sec- 
tions prepared  by  Dr.  Mott.) 


*  Pitres,  'Arch,  de  Physiologic,'  1874;  Hadden  and  Sherrington,  'Brain,*  Jan., 
1886.  An  illustration  of  this  bilateral  degeneration  will  be  found  in  the  section  on 
Diseases  of  the  Brain,  Vol.  II. 

t  Muratoff,  '  Neur.  Centralbl..'  1893 ;  Melius, '  Proc.  Royal  Society,*  1894  j  Sher. 


212  SPINAL   COKD. 

ruption  to  the  motor  path,  and  they  are  weakened  on  the  unparalysed 
side.  The  subject  is  considered  further  in  Vol.  II.  On  the  whole, 
the  facts  hitherto  ascertained  suggest  that  the  arrangements  which 
subserve  this  relation  vary  in  different  persons,  just  as  does  the 
chief  decussation  of  the  pyramids.  The  fibres  probably  in  some 
cases  continue  downwards  on  the  same  side  until  they  reach  the  level 
of  grey  matter  for  which  they  are  destined.*  In  other  cases  most  or 
many  of  them  cross  at  the  bulbar  decussation  Lastly,  there  is  the 
possibility  that  a  connection  between  the  motor  cells  or  structures  of 
the  two  sides  may  supplement  the  decussation.f 

How  do  the  fibres  of  the  pyramidal  tract  end  ^  They  seem  all  to 
pass  into  the  intermediate  grey  matter,  and  forwards  into  the  anterior 
cornu,  among  the  motor  nerve- cells.  There  they  are  lost  in  the 
plexus  of  the  spongy  substance.  There  is  strong  indirect  evidence 
that  they  divide  and  subdivide,  and  that  their  ramifications  come 
into  relation  with  the  plexus  of  nerve-fibrillse  constituted  by  the 
dividing  processes  of  the  ganglion-cells.  The  evidence  of  this  is 
twofold.  As  far  as  is  at  present  kuown,  only  one  process  of  a 
ganglion-cell  becomes  an  axon;  the  others  divide.  The  axon  passes 
into  an  anterior  root;  it  can  be  seen  to  do  so  in  the  case  of  some 
ganglion-cells  (Fig.  68),  and,  as  we  have  seen,  the  number  of  ganglion- 
cells  and  anterior  root-fibres  is  nearly  the  same  in  the  frog,  and 
therefore,  presumably,  in  man  also.  Since  some  root-fibres  are  not 
motor,  the  number  of  motor  cells  may  safely  be  assumed  to  be  at 
least  not  smaller  than  that  of  the  motor  fibres ;  thus  each  fibre  will 
have  at  least  one  cell  related  to  it.  If  so,  since  the  pyramidal  fibres 
are  certainly  connected  with  the  ganglion-cells,  the  connection 
can  only  be  by  the  dendrons  or  ramifying  processes  of  the  cells, 
and  this  involves  a  division  of  the  nerve-fibre.  The  dividing  fibre 
isolated  by  Gerlach,  and  shown  in  Fig.  69,  may  have  been  a  branch 
of  a  pyramidal  fibre.  The  second  point  in  evidence  of  division  is 
the  fact  that  the  nerve-cells  and  root-fibres  must  be  many  times 
more  numerous  than  the  fibres  of  the  pyramidal  tract. J  But  all 
(or  at  least  most)  of  the  motor  root-fibres  may  be  stimulated  through 
the  pyramidal  fibres,  and  therefore  each  of  the  latter  must  be  con- 
nected with  several  ganglion-cells.  Physiological  considerations  sug- 
gest the  same  conclusion,  which  is  fully  confirmed  by  the  new  histo- 

rington, 'Lancet,'  1894,  vol.  i ;  Rothmann,  'Neur.  Centralbl.,'  1896;  Redlioh,  ibid., 
1897.  Their  results,  obtained  by  the  method  of  degeneration,  have  been  further 
boine  out  by  the  ingenious  experiments  of  Wertheimer  and  Liepage  ('Arch.de 
Physiol.,'  1897). 

•  Dejerine  and  Thomas  have  traced  homolateral  fibres  as  low  as  the  4tii  sacral 
root  ('Compt.  Rend.,'  loc.  cit.),  Russell  to  5th  root  ('  Brain,'  loc.  cit.). 

f  Broadbent,  Hadden.  In  a  unilateial  lesion  of  the  cord  the  paralysed  foot  may 
move  with  the  other.  Such  a  connection  of  nuclei  was  originally  suggested  by 
Broadbent  to  explain  the  association  of  bilateral  movements. 

J  The  total  area  of  the  anterior  roots  of  the  cord  is  probably  at  least  five  timea 
as  great  as  that  of  the  pyramidal  tracts  in  the  upper  cervical  region. 


STRUCTURE. 


213 


logical  methods  to  which  reference  has  ah-eady  been  made.*     It  is  a 

curious  fact  that  the    degeneration    method   of 

Marchi  does  not  reveal  this  wholesale  passage  of 

the   pyramidal   fibres   into   the   anterior   coruua. 

This  is  probably  due  to  their  passing  in  in  the 

form  of  non-medullated  collaterals. 

Let  us  now  consider,  for  a  moment,  the  whole 
motor  path,  from  the  cortex  of  the  brain  to  the 
muscles.  We  may  regard  it  as  composed  of  two 
neurons,  an  upper  and  lower  (Fig.  74).  Each 
consists  of  a  ganglion-cell  above,  an  axon,  and  the 
terminal  ramification  of  the  latter.  The  upper, 
"  cerebro- spinal  "  neuron  consists  of  the  cortical 
ganglion-cell  with  its  dendrons,  and  the  "  pyra- 
midal" axon  which  proceeds  from  the  cell,  passes 
through  the  brain  and  cord,  and  ends  in  the 
grey  substance  by  division  and  terminal  inter- 
lacement with  several  nerve-cells.  The  lower, 
"  spino-muscular "  neuron  consists  of  the  spinal 
motor  cell  with  its  dendrons  and  the  axon  jjro- 
ceeding  from  it,  which  passes  through  the  an- 
terior root  and  nerve-trunk  to  the  muscle,  where 
it  divides  and  ramifies  on  the  muscular  fibre. 
The  elements  of  the  two  neurons  do  not  corre- 
spond in  number,  since,  as  we  have  just  seen, 
each  cerebro- spinal  element  is  connected  with  '/f/'/rv}'/\i  \]\' 
many  spino-muscular  elements.  So,  too,  each  '^^^^'^L^  ^I-^tJ^^y 
motor  axon  is  connected  with  a  considerable  area 

*  Gad  and  Flatau  ('Neurol.  Centralbl.,'  1897,  p.  481) 
h;>Te  endeavoured  to  mark  out  the  localisation  within  the 
motor  tracts  of  the  fibres  for  different  parts  of  the  body. 
Their  method  consisted  in  cutting  across  the  cords  of  dogs 
and  immediately  wpplying  to  different  parts  of  the  cross- 
section  of  the  lower  segment  stimuli  of  varying  strength 
and  duration.  They  found  that  in  general  the  fibres  nearest 
to  the  anterior  grey  matter  supplied  the  body  at  or  near 
the  level  of  the  section,  while  those  fur  other  regions  ran 
more  posteriorly  and  superficiiilly.  This  is,  however,  only 
what  would  follow  from  the  fact  that  the  axons  of  the 
upper  neuron  (».  infra)  terminate  by  interlacing  with  the 
dendrons  of  the  gangiion-cells  in  the  anterior  cornna,  from 
which  the  lower  axons  to  the  region  supplied  by  the 
segment  Hrise. 

Fig.  74. — Diagram  of  an  element  of  the  motor  path.  Show- 
ing cortical  cell,  pyramidal  fibre,  anterior  horn  cell,  nerve- 
fibre,  and  muscle.  More  than  one  anterior  horn  cell 
should,  strictly  sneaking,  be  shown  coming  into  relation 

with  the  terminal  ramifications  of  one  pyramidal  fibre.     To  show  this,  however, 
would  have  needlessly  complicated  the  diagram. 


214 


SPINAL  COED. 


C3 

Piff.  75.  —  Dei,'eiieration  of  the 
anterO'Iateral  ascending  tract  in 
a  case  of  locomotor  ataxy  (q.  v, 
figure),  showing  its  position  in 
the  upper  cervical  region,  where 
the  direct  cerebellar  tract  passes 
outside  it  into  the  restif  orm  body. 


FiQ.  76. — Ascending  defene- 
ration in  thepostero-inedian 
column  and  antero-Literal 
ascendino;  tract.  The  cord 
was  crushed  at  the  first  lum- 
bar segment. 


Db 


m^   Dc 


La 


L\. 


Fig.  77.-D'jgeneratiou  aftei 
injury  to  tlie  cauda  equina. 
C  a,  b,  c,  upper  middle  and 
lower  cervical;  Da,  upper; 
b,  c,  lower  dorsal ;  L  a, 
upper;  and  L  b,  middle  lum- 
bar re<rion.  (After  Scliultze^) 


of  excitable  muscle-tissue.     It  will  be  found  that  this  conception  of  the 
motor  path  conduces  to  clearer  ideas  of  many  facts  of  disease,  and  it  is 


STRUCTURE.  215 

important  to  grasp  it  firmly.  We  shall  see,  for  instance,  that  diseases 
involving  any  part  of  a  neuron  produce  similar  effects,  however  diverse 
their  nature;  while  there  is  a  fundamental  difference  between  the 
effects  of  disease  of  the  two  neurons.* 

Fibres  that  degenerate  downwards  through  the  greater  part  of  the 
cord  are  also  scattered  through  the  antero-lateral  column,  in  a  zone 
that  extends  beneath  the  surface  between  the  direct  pyramidal  tract 
and  the  direct  cerebellar  tract.  Some  are  mingled  with  the  fibres 
of  the  ascending  antero-lateral  tract  presently  to  be  described  ;  others 
lie  beneath  this.  It  is  possible  that  they  really  belong  to  the  crossed 
pyramidal  tract.  They  have  been  termed  by  Foster  the  descending 
antero-lateral  tract.f 

Bechterew  has  pointed  out  that  the  crossed  pyramidal  tracts  as 
mapped  out  by  the  degeneration  and  embryological  methods  do  not 
correspond.  In  other  words,  the  area  of  non-myelinated  nerve-fibres 
found  at  birth  is  much  greater  than  that  degenerating  as  the  result 
of  section  of  the  pyramidal  tract  high  up.  Boyce  and  Sakowitsch 
have  shown  independently  that  the  axons  of  the  remaining  area — 
which  is  situated  at  the  anterior  end  of  the  crossed  pyramidal  tract 
— descend  from  the  posterior  corpora  quadrigemina.J 

Fibres  degenerate  upwards  in  both  the  lateral  and  the  posterior 
columns.     We  may  consider  the  latter  first. 

Each  posterior  column,  as  we  have  seen,  is  divided  by  the  imperfect 
"intermediate  septum"  into  two  parts, — a  narrow  " postero-median 
column,"  and  a  wider  "  postero-external  column"  (Fig.  65).  In  the 
lumbar  and  sacral  regions  it  is  not  possible  to  separate  the  postero- 
external from  the  postero-median  column,  as  can  be  done  in  the  dorsal 
and  cervical  regions.  The  differentiation  is  not  completed  below  the 
level  of  the  eleventh  dorsal  segment.     Secondary  degeneration  con- 

*  This  conception  of  the  motor  path  was  published  in  its  present  form  (except, 
of  course,  that  the  neuronic  nomenclature  had  not  then  been  intrciduced)  in  the  first 
edition  of  this  book  in  August,  1886.  By  a  curious  coincidence  nearly  the  same  idea 
was  stated  (no  doubt  independently),  and  even  the  same  terminology  employed,  by 
Dr.  V.  Kenz  in  the  *  Centralblatt  f.  Nervenkr.,'  October  15th,  1886. 

t  Foster,  *  Physiology,'  1890,  p.  873.  See  also  Hadden  and  Sherrington,  'Brain/ 
vol.  viiij  and  Tooth,  '  Secondary  Degeneration  of  the  Spinal  Cord,'  1889.  Luciani 
and  Marchi  state  that  this  tract  is  mainly  composed  of  axons  descending  from  the 
cerebellum,  a  view  which  is  upheld  by  Biedl  ('  Neurol.  Centralbl.,'  1895).  Russell 
denies  this,  and  states  that  these  axons  are  derived  mainly  from  Deiter's  nucleus 
in  the  medulla,  a  view  with  which  Mott  agrees.  Held  has  shown  that  fibres  derived 
from  the  red  nucleus  pass  into  the  lateral  column  of  the  cord  ('Archiv  f.  Anur,. 
u.  Physiol.,'  1892). 

X  See  Bechterew,  *  Neurol.  Centralbl.,'  1897,  No.  23,  where  a  tract  in  the  anterior 
ground  bundle  descending  from  the  anterior  corpora  quadrigemina  is  also  de- 
scribed.  The  terra  « retrograde  degeneration "  has  been  applied  by  Sottas  and 
others  to  an  ascending  change  which  occurs  in  certain  cases  in  the  pyramidal  tracts. 
This  degeneration  is  slow  in  developing,  and  undergoes  gradual  diminution  in 
intensity  and  extent  as  it  ascends.  A  similar  condition  has  been  found  in  the 
posterior  columns  after  cerebral  lesions  (Durante,  'Rev.  ueurologique,'  J 898),  and  in 
tlie  direct  cerebellar  tract  after  a  cerebellar  lesion  (Campbell,  '  Brain,'  1897). 


216 


SPINAL   COED. 


firms  tMs  distinction.  If  the  posterior  columns  are  interrupted 
anywhere  in  their  course,  ascending  degeneration  results;  and  this, 
a  short  distance  aboye  the  lesion,  is  confined  to  the  posterior  median 
column  (rigs.  72,  76,  77),  in  which  the  degeneration  contmuea 
upwards  to  the  medulla  oblongata,  and  ends  there  at  the  grev 
matter  of  the  "nucleus  gracilis."  The  fibres  of  these  median 
columns  degenerate  in  the  same  way  when  the  lesion  is  not  in 
the  cord,  but  in  the  posterior  nerve-roots  (as  of  the  cauda  equina, 
Fig.  77).  Hence  it  is  clear  that  these  fibres  are  continued  upwards 
from  the  posterior  nerve-roots  without  interruption.    The  contrast 


Fia.  78. 


Fio.  79. 


Pia.  78. — Degeuerations  after  a  lesion  of  the  cord  in  the  lower  part  of  the  cervical 
redon.  A,  highest  cervical ;  B,  middle  cervical  li  cm.  ahove  lesion.  Degtneration 
of^the  posterior  median  columns,  spreading  slightly  in  A  and  widely  in  b  into  the 
})0st.  ext.  col.  The  nature  of  the  circumferential  degeneration  is  uncertain;  it 
IS  probably  a  partial  ascending  degeneration  set  up  by  peripheral  myelitis. 

C,  upper  dorsal  2  cm.  below  lesion.  Degeneration  of  the  lateral  pyramidal  tracts; 
"  co'mma-shaped "  degeneration  in  front  of  the  post.  ext.  col. ;  slight  degenera- 
tion of  the  anterior  ground-fibres.     (Schultze.) 

FlO-.  79. — Ascending  degeneration  after  injury  to  the  lowest  part  of  the  spinal 
cord  and  sciatic  nerve-roots  arising  from  it.  A,  cervical ;  B,  lower  dorsal  j  c,  junction 
of  dorsal  and  lumbar  regions  ;  d,  middle  of  lumbar  enlargement.     (After  Schultze.) 

between  these  degenerated  columns  and  the  undegenerated  external 
columns  through  the  greater  part  of  the  cord  above  the  lesion  (Fig.  76) 
is  very  striking.  But  just  above  the  lesion  tlie  degeneration  is  not 
confined  to  the  median  columns  ;  it  spreads  out  into  the  external 
columns,  especially  towards  the  hinder  surface,  and  the  more  exten- 
sively the  nearer  to  the  lesion,  until  close  above  this  the  degenerated 
fibres  occupy  the  whole  extent  of  the  posterior  column,  except  a  small 
area  close  to  the  posterior  cornu.     This  is  due  to  the  fact  that  the 


STRUCT UKB.  217 

fibres  pass  to  the  median  column  tlirougli  tlie  hinder  part  of  the 
external  column.  It  is  the  same  whatever  is  the  seat  of  the  lesion, 
whether  it  is  in  the  cervical  (Fig.  78),  dorsal,  or  lumbar  (Fig.  79) 
parts  of  the  cord ;  and  if  it  is  in  the  cauda  equina,  the  degeneration 
spreads  out  in  the  same  way  in  the  lumbar  enlargement  (Fig.  7*?). 
This  shows  clearly  that  all  the  way  up  the  cord  fibres  pass  to  the 
median  column  from  the  external  column,  and  that  these  are  uncrossed 
fibres  from  the  posterior  roots.*  As  the  fibres  ascend  the  cord  in  the 
median  column,  they  come  to  He  posteriorly  in  proportion  as  their 
som-ce  is  lower.  Those  from  the  sciatic  nerves,  for  instance,  m.  the 
lower  cervical  cord  only  occupy  the  posterior  half  of  the  columns 
(Fig.  79).  Those  from  the  whole  himbar  enlargement,  or  whole 
cauda  equina  (Fig.  7&),  reach  forward  to  the  commissure  in  the  lower 
cervical  region,  spreading  out  a  little  near  the  commissure,  where, 
however,  the  degeneration  is  much  less  dense  than  it  is  behind 
Above  the  middle  of  the  cervical  enlargement  the  degeneration  from  a 
lesion  in  the  dorsal  or  lumbar  regions  does  not  extend  forwards 
beyond  the  junction  of  the  anterior  and  middle  third  of  these 
columns ;  but  if  the  lesion  is  in  the  cervical  region  the  degeneration 
extends  up  to  the  commissure,  even  at  the  level  of  the  second  cervical 
segment  (Fig.  78,  a),  showing  that  the  fibres  from  the  cervical  roots 
occupy  the  anterior  portion  of  these  columns.  It  is  now  known 
that  most  if  not  all  root-fibres  divide  on  entering  the  posterior 
columns  into  ascending  and  descending  branches  With  the  course 
of  the  former  we  have  just  been  occupied  ;  the  latter  run  but  a  short 
way  down  in  the  cord  and  terminate  by  turning  into  the  posterior 
cornu  and  inosculating  with  the  cell-dendrites  there  found. 

It  is  important  to  remember  that  the  root-fibres  that  pass  to  the 
median  column  through  the  external  column  do  not  decussate.  This 
is  not,  however,  the  only  source  of  fibres  to  the  median  column. 
Fibres  pass  to  it  (1)  from  the  neck  of  the  posterior  horn,  across  the 
anterior  part  of  the  external  column,  curving  backwards  (Fig.  80,  x)  ; 
many  of  them  extend  almost  to  the  postero-median  septum.  They 
are  "  endogenous,"  i.  e.  they  arise  from  cells  of  the  posterior  cornua. 
In  the  posterior  columns  they  bifurcate  into  ascending  and  descending 
portions,  the  former  of  which  may  in  certain  parts  of  the  cord  form 
short  but  definite  tracts,  such  as  the  "comma"  tract  of  Schultzef  and 
that  in  the  lumbo-sacral  region  referred  to  below.  These  descending 
fibres  termimite  in  all  probability  by  again  turning  into  the  grey 
matter  of  the  posterior  cornua.  (2j  From  the  posterior  commissure  in 
the  middle  line  (Fig.  80).  These  are  very  numerous  and  conspicuous 
in  tbe  lumbar  enlargement.     They  course  backwards  in  the  septum 

*  Except  in  cases  in  wliich  the  cervical  roots  are  affected  in  which  degeneration 
is  restricted  to  the  postero-extenial  column  terminating  in  the  cuneate  nucleus. 
Goinbault,  'Bull,  de  la  Soc.  d'Anatomie,'  1891;  Sottas,  'Rev.  de  Med,,'  1893; 
Dejerine  and  Thora:is,  '  Compt.  Rend.,'  1896;  Souquez,  '  Compt.  Rend.,'  1895; 
Russell,  'Brain,'  1896. 

t  'Arch.  f.  Pliysiol.,' 1883. 


218  SPINAL    CORD. 

and  tlien  pass  outwards  on  each  side  into  the  adjacent  column. 
They  come  from  the  posterior  and  lateral  columns  of  the  opposite 
side,  decussating  in  the  septum.  Some  arise  from  posterior  root- 
fibres,  others  from  cells  in  the  posterior  cornua. 

In  the  lumbo-sacral  region  there  is  a  narrow  tract  of  fibres  close  to 
the  posterior  median  septum,  that  is  apparently  of  different  nature 
from  the  rest  of  the  column.  It  is  lenticular  on  transverse  section, 
and  is  indicated  by  the  clear  area  in  l.  4,  Fig.  71,  and  faintly  in  L  b. 
Fig.  *1*1  \  it  is  best  seen  from  the  third  lumbar  to  the  second  sacral 
segments.  It  is  distinguished  by  a  difference  in  time  of  development 
(Flechsig),  and  by  freedom  from  the  secondary  degeneration  that 
involves  the  rest  of  the  column.  It  has  been  observed  to  undergo 
descending  degeneration  in  a  certain  number  of  cases,  and  its  fibres 
appear  to  terminate  in  the  posterior  horn  of  the  same  side.  Their 
orio-in  is  uncertain,  but  they  are  probably  endogenous  and  not  directly 
connected  with  the  posterior  root.*  A  carefid  study  by  Pineles  has 
shown  that  they  fail  to  degenerate  in  tabes,  and  they  also  escape  in  th^ 
degeneration  associated  with  anaemia. f  This  tract  is  possibly  identical 
with  Flechsig's  "  oval  field,"  and  is  .also  known  as  the  septo -marginal 
tract  (seep.  220). 


Fl&.  80.— P.isferior  cornu  and  column  at  the  last  dorsal  segment,  p.  M.  0.,  postero- 
median column;  P.  E.  C,  postero- external  column;  P.  M.  s.,  posterior  median  sep- 
tum J  p.  c,  posterior  commissure ;  v,  commissure  vein.  P.  V.  c,  posterior  vesicular 
column;  0.  c,  caput  coriiu ;  P.  E.,  posterior  root;  a,  an  artery;  d,  d,  d,  adjacent 
to  a  strip  of  the  lateral  column,  indicate  the  tracts  of  fibres  passing  from  the 
vicinity  and  interior  of  the  posterior  vesicular  column  along  the  septa  of  the 
lateral' column,  to  form  the  direct  cerebellar  tract;  a;,  a?,  tracts  of  fibres  passing 
from  the  neck  of  the  horn,  near  the  })0st.  vesic.  col.,  to  the  post-median  column. 

*  See  Hoche,  'Neurol.  Centralbl.,'  1896;    liruce  and  Muir,  '  Brain,'  1896. 
t  Tor  an  illustration  of  this  tract  see  later,  where  the  degenerations  associated 
with  anaemia  are  referred  to. 


STRUCTDRE.  219 

It  is  very  doubtful  whether  all  the  fibres  that  enter  the  postero- 
median columns  continue  in  these  to  the  medulla.  Some  certainly  do, 
but  the  upward  increase  in  size  of  the  columns  seems  to  be  far  too 
small  for  the  accommodation  of  all  the  fibres  that  seem  to  pass  to  them. 
At  the  same  time  we  have,  at  present,  no  indication  of  the  mode  in 
which  fibres  leave  these  columns. 

At  the  meduUa  oblongata  this  column  becomes  filled  with  nerve- 
cells,  the  post-pyramidal  nucleus,  so  called  because  the  highest  por- 
tion of  this  column  has  been  termed  the  posterior  j/yramid  of  the 
medulla.  It  is  also  called  the  nucleus  gracilis.  The  discovery  that 
the  fibres  of  this  column  are  continuous  with  the  nerve-roots  invests 
the  post-pyramidal  nucleus  with  considerable  importance,  since  its 
nerve-cells  are  the  first  with  which  these  root-fibres  are  related,  and 
secondary  degeneration  hence  stops  here.  The  upward  degeneration  of 
the  median  fibres  implies  upward  conduction  ;  their  probable  function 
wiU  be  considered  in  the  next  section.  It  is  now  known  that  not  all  of 
them  end  in  the  gracile  nucleus ;  some  enter  into  the  external  arcuate 
fibre-system,  while  others  run  in  the  restiform  body  to  the  cerebellum. 

The  postero-extemal  column  {"  column  of  Burdach,"  containing  the 
"  posterior  root-zone "  of  Charcot)  consists  chiefly  of  vertical  fibres. 
Many  of  the  posterior  root-fibres  pass  through  it,  horizontally  or 
obhquely  (Fig.  65).  These  either  curve  inwards  to  the  posterior 
horn  (some  first  inclining  upwards),  or  pass  obliquely  upwards  and 
inwards  to  the  median  column  and  constitute  the  fibres  of  this  column 
that  are  continued  from  the  nerve-root.  Across  the  anterior  part 
fibres  also  pass,  as  just  described,  between  the  neck  of  the  posterior 
horn  and  the  median  column.  The  vertical  ground- fibres  of  the 
external  column  have  apparently  only  a  short  coiirse,  since  they 
degenerate  for  only  a  few  centimetres  above  or  below  a  lesion.  The 
longest  descending  degeneration  is  of  a  "  comma- shaped  tract,"  in 
the  middle  of  the  anterior  third  of  the  column  in  the  dorsal  region, 
which  may  degenerate  dovniwards  for  eight  or  ten  segments  *  (Fig.  78). 
These  vertical  fibres,  of  short  course,  may  connect  the  grey  matter  of 
the  posterior  horn  at  different  (but  adjacent)  levels;  this  is  rendered 
probable  by  the  fact  that  they  degenerate  in  transverse  lesions  of  the 
cord  but  not  in  those  of  the  posterior  roots.f  This  column  is  larger 
in  the  swellings  than  it  is  in  the  dorsal  region,  chiefly  in  consequence 
of  the  larger  number  of  root-fibres  that  pass  through  it.  Above,  it  also 
ends  in  a  grey  nucleus,  the  posi era- external  nucleus  or  cuneate  nucleus. 

Both  these  posterior  nuclei  are  connected  directly  with  the  cortex 
of  the  opposite  hemisphere,  and  chiefly  with  the  ascending  parietal 
convolution.     Fibres  also  pass  from  them  to  the  cerebellar  hemisphere 

•  Hoclie,  'Arch.  f.  Psych,  u.  Nervenkrank.,'  xxvii. 

+  Gombanlt  and  Philippe,  'Sem.  Med.,'  1894.  This  is  only  true  of  experimenfcil 
division  of  the  posterior  roots,  not  of  two  cases  described  by  Dejerine  and  Thomas 
('Compt.  Rend.,'  189G),  and  Nageotte  (' Uev.  Neurolojjique,' 1895),  in  whi<h  the 
degeneration  was  found  to  extend  down  the  cord.  See  also  Eussell  ('  Brain/ 
Summer,  1898). 


220  SPINAL    CORD. 

of  their  own  side,  wMcli  is  connected  witli  the  same  region  of  tlie  opposite 
cerebral  cortex,  probably  through  the  red  nucleus  and  optic  thalamus.* 
The  two  nuclei  (of  both  sides)  are  also  found  (by  developmental  re- 
searches) to  send  fibres  through  to  the  inferior  cerebellar  peduncle,t 
and  fibres  pass  from  the  posterior  median  nucleus  to  the  middle  lobe 
of  the  cerebellum  on  the  same  side,  J  a  fact  of  considerable  signi- 
ficance. Two  other  tracts  of  endogenous  fibres,  i.  e.  fibres  not  derived 
from  the  roots  but  from  cells  in  the  grey  matter  of  the  cord  itself, 
have  been  described  in  the  posterior  columns — one  by  Marie,  the 
cornu-commissural  tract,  in  close  relation  to  the  posterior  commissure 
throughout  the  sacral  and  lumbar  regions  of  the  cord;  the  other, 
which  has  been  named  the  sejpto -marginal  tract  (see  p.  218),  is  in 
proximity  to  the  septum  as  high  as  the  eleventh  dorsal  segment. 
Some  observations  would  seem  to  indicate  that  it  may  originate  as  high 
as  the  cervical  regions.  §  These  tracts  remain  undegenerated  in  tabes 
dorsalis,  and  in  the  degenerations  associated  with  anaemia  (p.  218). 

The  "  direct  or  dorsal  cerebellar  tract  "  is  another  series  of  fibres  that 
degenerate  upwards.  It  forms  a  layer  at  the  periphery  of  the  lateral 
column,  outside  the  pyramidal  tract  (Fig.  65),  but  does  not  extend 
through  the  whole  length  of  the  cord,  ceasing  below  at  the  level  of  the 
first  lumbar  nerve  (Fig.  71).  The  anterior  part  of  the  tract  (as  seen 
in  section)  does  not  extend  forwards  beyond  the  level  of  the  lateral 
pyramidal  tract,  although  it  was  formerly  thought  to  do  so  because 
there  are  other  fibres  in  front  of  it  that  also  degenerate  upwards, 
and  these  were  not  distinguished  from  those  of  the  tract.  But  near 
its  upper  and  lower  extremities,  at  the  level  of  the  second  cervical 
nerves,  and  also  in  the  lowest  part  of  the  dorsal  region,  the  tract  lies  a 
little  anterior  to  its  position  in  the  rest  of  the  cord,  and  hence  at  these 
places  the  pyramidal  tract  comes  to  the  surface  behind  the  cerebellar 
tract,  close  to  the  posterior  nerve-roots,  up  to  which  elsewhere  the 
cerebellar  tract  extends.  The  tract  increases  somewhat  in  size  from 
below  upwards,  and  hence  receives  fibres  throughout  its  course,  but 
most  of  its  constituent  fibres  enter  it  at  the  level  of  the  lowest  dorsal 
and  first  lumbar  nerves,  i.  e.  at  its  lowest  part.  These  fibres  come 
from  the  grey  substance,  passing  through  the  lateral  column,  along 
the  septa  that  cross  the  pyramidal  tract  from  the  grey  matter.  In  the 
latter  these  fibres  are  conspicuous  objects  at  this  level  (Fig.  80,  d,  d), 
passing  transversely  and  obliquely  from  the  front  of  the  posterior 
vesicular  column.  Into  this  many  of  them  can  be  traced ;  others 
change  their  direction  and  become  vertical,  perhaps  passing  into  the 
vesicular  column  at  a  different  level.  It  is  now  known  that  the  fibres 
come  from  this  column ;  the  cerebellar  tract  is  chiefly  formed  at  that 
part  of  the  cord  at  which  the  column  is  most  developed,  and  the  cells 

*  See  Flechsig  and  Hosel,  'Neurol.  Cent.,'  1890,  p.  417. 
t  Darkschewitsch  and  Freud,  ib.,  1886,  p.  121. 
X  Bechterew,  ♦  Wjistnik  Psych.,  &c.,'  1886. 
§  A.  Bruce,  'Brain,'  Autumn,  1897. 


STRUCTUEB.  221 

of  this  column  have  been,  found  atrophied  when  the  cerebellar  tract  is 
degenerated.*  Moreover  modern  staining  methods  have  demonstrated 
the  origin  of  the  axons  of  the  direct  cerebellar  tract  from  the  cells  of 
Clarke's  column.  It  has  also  been  shown  that  axons  pass  to  this 
tract  from  Stilhng's  sacral  nucleus,  which  is  the  sacral  homologue  of 
Clarke's  column.  Besides  the  fibres  that  are  gathered  into  the  com- 
pact cerebellar  tract,  others,  apparently  belonging  to  it,  ascend  among 
those  of  the  adjacent  pyramidal  tract. 

The  cerebellar  tract  only  degenerates  when  a  lesion  of  the  cord  is  at, 
or  above,  its  place  of  origin,  the  junction  of  the  dorsal  and  lumbar 
regions  If  the  lesion  is  in  the  lumbar  enlargement,  as  in  the  case 
shown  in  Fig   76,  the  tract  does  not  degenerate. 

At  the  level  of  the  first  cervical  nerve  (where  the  pyramidal  tract 
leaves  the  lateral  column  to  cross  into  the  anterior  pyramid  of  the 
medulla)  the  cerebellar  tract  lies  in  front  of  the  "  grey  substance  of 
Rolando  "  (formed  from  the  caput  comu  posterioris),  and  passes  up,  in 
the  restiform  body,  to  the  cerebellum.  Since  it  degenerates  upwards 
we  must  conclude  that  it  also  conducts  upwards.f 

We  have  seen  that  the  lateral  pyramidal  tract,  although  in  contact, 
behmd,  with  the  head  of  the  posterior  cornu,  does  not  extend  quite 
up  to  the  neck  of  the  horn  or  to  the  intermediate  grey  substance, 
the  two  being  separated  by  a  "  lateral  limiting  layer  "  of  vertical  fibres, 
in  part  broken  up  by  processes  from  the  grey  matter  In  the  upper 
cervical  cord,  and  also  to  a  less  extent  in  the  dorsal  cord,  this  layer 
extends  forwards  outside  the  anterior  horn,  which,  in  these  parts,  is 
narrow  It  consists  of  fine  fibres  that  seem  to  pass  into  and  out  of 
the  grey  matter.  This  course,  and 
the  fact  that  the  tract  does  not  dege- 
nerate through  any  considerable  ex- 
tent, suggest  that  its  fibres  have  but 
a  short  course,  and  connect  the  grey 
matter  of  adjacent  regions. 

There  is  a  small  tract  of  fibres,  all 
of  smaU  size,   at   the   junction  of  the  ^'^-  f— Cervical  section.     As. 

'         .  ••  cending  degeneration  in  post- 

tip  of  the  posterior  horn  and  lateral  med.col  and  ant.-lat.  ascending 

column    known    as   Lissauer's   tract.  ^^^^^'  secondary  to  a  lesion  in 

mn        r;i  £  ±1  ,      •  t^^    dorsal    region.     (From    a 

The  fibres   come  from  the  posterior  gection  prepared  by  Dr.  Mott.) 

roots  ;  they  course  upward  for  a  short 
distance,  forming  the  tract,  and  then  enter  the  posterior  horn. 

*  H.  g.  by  Minkowski,  *  Deut.  Arch.  f.  kl.  Med.,'  Bd.  xxxiv,  p.  433.  Slight  degene- 
ration of  the  pyramidal  tracts  was  the  only  other  lesion. 

t  In  the  absence  of  evidence  of  contrary  character,  the  direction  in  which  fibres 
conduct  must  be  assumed  to  be  that  in  which  they  degenerate.  We  know  of  no 
exceptions  to  it  within  the  central  nervous  system.  Wherever  we  can  observe  the 
relations  this  law  obtains,  except  in  the  peripheral  sensory  nerves,  in  which  the  con- 
ditions are  conspicuously  exceptional.  Hence  the  probability  is  very  great  that  the 
correspondence  obtains  where  we  have  not  yet  been  able  to  prove  it;  and  to  ignore 
the  probability  because  it  is  not  a  certainty  is  scarcely  reasonable,  although  not 
uncommon. 


222  SPINAL    CORD. 

Tlie  rest  of  tlie  lateral  column,  in  front  of  tlie  pyramidal  and  cere- 
bellar tracts,  consists  of  fibres  that  vary  in  size,  course,  and  date  of  de- 
velopment. Secondary  degeneration,  however,  as  I  pointed  out  some 
years  ago,*  enables  us  to  distinguish  an  important  tract  which  occupies 
an  irregular  area  ia  front  of  the  pyramidal  and  cerebellar  tracts,  and 
degenerates  upwards  throughout  the  cord.  It  extends  across  the  lateral 
column,  as  a  band  which  fills  up  the  angle  between  the  pyramidal  and 
cerebellar  tracts,  and  it  reaches  the  surface  of  the  cord  in  front  of  the 
latter  tract,  nearly  on  a  level  with  the  anterior  commissure ;  it  then 
extends  forward  in  the  periphery  of  the  anterior  column,  almost  to  the 
anterior  median  fissure,  and  up  to  the  direct  pyramidal  tract  when  this 
exists.  I  have  termed  it  the  antero-lateral  ascending  tract  (Figs.  65, 
71,  75,  76,  and  81).  It  has  often  been  confounded  with  the  direct 
cerebellar  tract,  and  hence  the  impression  arose  that  the  latter  extends 
farther  forwards  than  it  really  does.  Posteriorly  the  tract  extends  across 
the  lateral  column  towards  the  posterior  commissure  (Fig.  71,  l.  4). 
In  the  cervical  region  (ib.,  c.  3),  especially  ia  the  upper  part,  where 
the  direct  cerebellar  tract  extends  farther  forward,  the  broadest  part 
of  the  ascending  tract  lies  just  within  the  anterior  part  of  the  cere- 
bellar tract,  and  extends,  like  a  wedge,  between  the  latter  and  the 
pyramidal  tract. 

This  tract  has  been  found  by  Bechterew  to  undergo  development  at 
a  different  period  from  the  rest  of  the  lateral  column.  It  apparently 
constitutes  an  upward  path  from  the  opposite  posterior  roots,  since  its 
degeneration,  if  unequal  on  the  two  sides,  is  greater  on  the  side  oppo- 
site to  that  on  which  the  uncrossed  postero-median  column  is  most 
degenerated.  Its  fibres  do  not  degenerate  when  the  nerve-roots  are 
divided,  and  hence  probably  spring  from  cells  in  which  the  root- 
fibres  end.  Fibres  apparently  pass  to  it  through  the  whole  length  of 
the  cord,  and  these  are  mingled  together,  so  that  the  degeneration 
from  a  lesion  of  the  lower  part  of  the  cord  is  not  dense.f  The  precise 
form  of  the  tract  seems  subject  to  individual  variations.  Above,  its 
fibres  probably  end  in  the  cerebellum.^ 

*  '  Diagnosis  of  Diseases  of  the  Spinal  Cord,'  first  ed.,  1879. 

f  Other  illustrations  of  the  degeneration  of  this  tract  will  be  found  in  the  section 
on  Locomotor  Ataxy.  For  an  example  of  its  degeneration  and  references  to  pre- 
vious observations,  see  Tooth, '  St.  Bartholomew's  Hospitnl  Reports,'  1885,  p.  137. 
See  also  his  '  Lectures  on  Secondary  D.  generation  in  the  Spinal  Cord,'  Lond.,  1890. 

X  Experiments  on  monkeys  show  that  the  antero-lateral  ascending  tract  as  here 
described  is  distinguishable  as  high  as  the  origin  of  the  fifth  cranial  nerve,  round 
which  it  curves.  It  then  passes  backwards  on  the  under  surface  of  the  superior 
cfirebellar  peduncle  to  reach  the  superior  vermis.  This  portion  is  now  known  as  the 
ventral  cerebellar  tract,  in  contradistinction  to  the  direct  or  dorsal  cerebellar  pre- 
viously mentioned.  (See  Mott,  *  Monatsschrif t  f.  Psych,  u.  Neurol.,'  i,  p.  104;  and 
van  Gehuchten,  'Anatomie  du  Syst&me  Nerveux,'  2nd  ed.,  p.  764  his.)  Mott 
('Brain,'  1895)  states  that  the  tract  can  be  distinguished  after  a  lesion  involving 
the  3rd,  4th,  and  5th  lumbar  segments  in  the  monkey.  Russell  ('  Brain,*  1898) 
from  pathological  evidence  confirms  this.  External  to  tbis  antero-lateral  ascending 
tract,  at  the  extreme  periphery,  is  a  tract  of  descending  fibres — the  efferent  or 


STliUCTURE.  223 

Some  other  fibres  are  also  found  in  the  position  of  the  antero- 
lateral ascending  tract,  constituting  the  "crossed  afferent  tract"  of 
E dinger.  They  probably  enter  from  the  posterior  root  and  come  into 
connection  with  cells  which  give  off  axis-cylinder  processes  decussating 
in  the  anterior  commissure.  They  pass  up  near  the  fillet  and  end  in 
the  corpora  quadrigemina,  or  optic  thalamus.* 

Some  fibres  of  the  anterior  part  of  the  lateral  column  are  large  in 
size,  and  curve  upwards  and  downwards  into  the  anterior  horn.  They 
must  be  of  short  course,  and  may  be  fibres  of  the  anterior  nerve-roots, 
which  ascend  and  descend  to  nerve-cells  on  a  different  level  from  that 
of  the  roots  by  which  they  leave  the  cord. 

The  fibres  of  the  anterior  column,  exclading  the  anterior  pyramidal 
tract,  are  termed  by  Flechsig  the  anterior  ground-fibres  (Fig.  65). 
They  are  not  separable,  structm-ally  or  by  development,  from  those  of 
the  lateral  limiting  layer.  The  ground-fibres  do  not  degenerate 
through  any  considerable  extent  of  the  cord,  and  some  probably  connect 
the  anterior  cornua  at  different  levels.  Moreover  some  of  the  fibres 
pass  to  the  anterior  commissure,  and  thus,  by  the  agency  of  the 
anterior  columns,  a  connection  may  be  established  between  the  two 
anterior  cornua  at  different  levels. 

The  commissure  of  the  spinal  cord  lies  between  the  bottom  of  the 
anterior  fissure  and  the  posterior  columns,  and  in  it  alone  is  there  a 
passage  of  fibres  from  one  half  of  the  cord  to  the  other.  It  consists 
of  two  parts,  an  anterior  or  white,  and  a  posterior  or  grey  commissure. 
The  white  commissure  varies  in  thickness  in  different  parts  of  the  cord, 
and  is  largest  in  the  lumbar  region.  It  is  composed  of  meduUated 
fibres,  which  cross  in  the  commissure  in  such  a  manner  that  the 
anterior  fibres  on  each  side  pass  out  in  the  posterior  pai-t  of  the 
commissure  on  the  other  side.  The  fibres  in  front  pass  into  the 
anterior  white  column,  those  behind  into  the  grey  substance;  hence 
they  appear  to  establish  a  connection  between  the  anterior  column  of 
one  side  and  the  anterior  cornu  of  the  other ;  but  many  of  those 
entering  the  anterior  column  merely  pass  through  this  to  the  cornu, 
and  this  is  probably  true  also  of  some  of  the  fibres  that  turn  upward 
in  the  column.  Some  seem  to  go  to  ganglion-cells  of  the  grey  matter 
or  to  the  fibrillary  plexus,  others  to  the  anterior  nerve-roots.  Through 
this  commissure  must  also  pass  the  fibres  of  the  anterior  pyramidal 
tract,  and  from  it  may  come  certain  fibres  that  have  been  traced 
across  the  intermediate  grey  substance  to  the  lateral  column,  where 
they  assume  a  vertical  direction  in  the  pyramidal  tract,  so  that 
part  of  the  anterior  commissure  is  to  be  regarded  as  a  continuation 

descending  antero-lateral  tract  referred  to  on  p.  215 — the  relations  of  which  are 
not  yet  quite  distinct  (see  Bieiil,  '  Neur.  Cent.,'  1895;  Mott,  '  Brain,'  1895;  Risien 
Russell, '  Brain,'  ISOSI. 

*  But  Rossolimo  ('  Neur.  Cent.,'  1898,  p.  935)  traces  Gowers's  antero-lateral  tract 
in  a  case  of  tumour  of  the  cord  into  the  posterior  corpoia  quadrig-einina,  the  sub- 
stantia nii-n  a,  and  the  gl  bus  pallidus.  Of  its  different  destination  in  monkeys  there 
seems  to  be  no  doubt. 


224 


SPINAL    CORD. 


,,^£^-^i  ' 


Y" 


of  the  decussation  of  the  pyramids.  Lastly,  a  few  fibres  of  the 
anterior  commissure  have  been  shown  to  come  from  the  posterior 
nerve- roots. 

The  grey  commissure  contains  very  fine  medullated  nerve-fibres,  a 
large  number  of  which  pass  backwards  in  the  posterior  horn  of  each 
side ;  some  appear  to  be  continuous  with  the  fibres  of  the  posterior 
roots,  while  others  probably  end  ia  the  nerve-ceUs  of  the  posterior 
horn.    It  is  highly  probable  from  clinical  evidence  that  a  considerable 

number  of  the  posterior  root-fibres 
(or  fibres  from  cells  in  which  the 
root-fibres  end)  cross  to  the  other  side, 
but  no  reliable  experimental  evi- 
dence of  such  a  crossmg  has  been 
pubHshed.  Within  the  grey  corn- 
commissure  is  the  central  canal  of  the 
spinal  cord,  lined  with  epithelium, 
and  surrounded  by  granular  material, 
said  to  be  similar  ia  nature  to  that 
which  caps  the  posterior  horn ;  the 
two  are,  indeed,  as  we  have  seen 
(p.  206),  continuous  in  the  embryo. 
The  canal  is  often  filled  up  by  epithe- 
lial debris  in  adult  cords,*  or,  some- 
times, is  much  larger  than  nomal. 
The  latter  state  probably  depends 
on  an  arrest  of  development,  for  the 
layer  of  neuroglial  tissue  around  it  is 
much  larger  than  usual,  f 

Root-fibres. — The  course  of  the  root- 
fibres  has  been  already  incidentally 
alluded  to,  but  remains  to  be  con- 
sidered in  a  more  systematic  manner. 
The  anterior  roots  pass  through  the 
anterior  columns  in  bundles  which  are 
distributed  through  an  area  of  the 
column  approximately  corresponding 
to  the  width  of  the  anterior  surface  of 
the  cornu.  In  the  grey  substance,  the 
coarse  fibres  that  constitute  the  chief  part  of  each  of  these  bundles 
pass  in  three  different  directions,  outwards,  inwards,  and  directly 


V^ 


Fia.  82. — Central  canal  ito  normal 
cords.  It  has  the  form  of  a 
transverse  slit  in  A,  a  vertical 
slit  in  C  (from  the  conus  medul- 
laris),  while  in  B  it  is  circula*. 
In  each  it  is  lined  by  columnar 
epithelium  and  surrounded  by 
cellular  elements,  rounded,  o* 
angular  from  compression 
mingled  with  granules.  In  D, 
which  is  from  the  same  spinal 
cord  as  C,  the  position  of  the 
canal  is  occupied  by  a  mass  of 
nuclear  tissue. 


•  This  obliteration  of  the  canal  has  no  pathological  significance,  but  has  been 
repeatedly  described  as  one  of  the  morbid  changes  in  disease. 

t  Ciaglinski  ('Neur.  Cent.,'  1896)  describes  a  long,  diamond-shaped  tract 
situated  in  the  grey  commissure  between  the  ventral  extremity  of  the  posterior 
columns  and  the  central  canal,  diminishing  in  volume  as  it  ascends,  but  traceable 
to  the  cervical  region.  He  believes  that  its  fibres  are  derived  from  the  posterior 
roots,  and  subserve  the  conduction  of  thern-a^  ftnd  painful  impressions. 


STRUCTURE.  225 

bactwards  in  tLe  middle  of  tlie  horn,  and  thns  cross  eacli  otlier  and 
the  fibres  from  the  anterior  commissure.  Many  of  the  fibres  enter 
the  groups  of  nerve-cells,  and  certainly  end  in  these  cells— or  rather 
arise  from  them,  each  axis-cyhnder  being  the  prolonged  chief  process 
of  the  cell.  Such  cells  often  lie  among  the  bundles  outside  the  limit 
of  the  grey  matter,  and  their  processes  can  easily  be  traced  forwards 
to  the  nerve-root  and  backwards  into  the  grey  matter.  Some  processes 
that  pass  outwards  enter  the  lateral  white  column  ("mixed  zone"), 
and  probably,  after  a  short  course  in  this,  re-enter  the  grey  matter  at 
a  higher  or  lower  level.  Some  of  the  fibres  that  pass  inwards  may  go 
through  the  commissure  to  the  opposite  anterior  horn.  These  coarse 
fibres  are  probably  all  motor.  Besides  them,  the  nerve-roots  contain 
fine  fibres  for  the  sympathetic  which  do  not  come  from  the  cornual 
cells. 

The  posterior  root-fibres  have  been  the  subject  of  much  investigation 
on  account  of  their  complicated  arrangement  and  the  importance  that 
attaches  to  them  from  their  degeneration  in  locomotor  ataxy.  Although 
arranged  in  a  contiauous  vertical  series  at  their  sm-face  attachment, 
the  fibres  are  divisible  into  three  sets,  one  of  which  passes  through 
the  caput  cornu  posterioris  (Fig.  80),  a  second  into  the  adjacent  part  of 
the  postero-external  column  (Fig.  65),  while  a  third  set  consists  of  fir.  .- 
fibres  which  at  once  change  their  direction,  turning  upwards,  and  keep 
together  so  as  to  form  a  zone  (zone  of  Lissauer), which  lies  outside  the 
extremity  of  the  posterior  horn  in  the  cervical  region,  but  lower  down 
the  cord  between  the  caput  and  the  surface  of  the  cord. 

The  fibres  of  these  three  sets  have  only  two  immediate  destinations — 
to  the  postero-median  column  and  the  posterior  grey  cornu.  (1)  Many 
of  the  fibres  that  enter  the  postero-external  column  (a)  pass  obhquely 
upwards  and  inwards  to  the  postero-median  column.  This  course 
is  through  the  hinder  part  of  the  postero-external  column,  and  in 
the  lumbar  region,  where  the  roots  enter  at  the  bottom  of  their  f ui-row, 
many  fibres  actually  pass  backwards  into  the  adjacent  angle  of  the 
column,  and  may  be  there  seen  to  turn  upward  in  their  ascending 
course  to  the  median  column.  It  is  this  arrangement  that  leads  to  the 
peculiar  wedge-like  shape  of  the  ascending  degeneration  just  above  a 
lesion  (Figs.  77  and  78)  ;  (b)  other  fibres  entering  the  postero-external 
column  curve  forwards  and  enter  the  posterior  cornu,  chiefly  in  front 
of  its  head.  Some  of  these  may  end  by  interlacing  with  the  processes 
of  cells  in  Clarke's  column  or  the  anterior  horns.  The  curves  they 
describe  are  largest  in  the  lumbar  region,  where  they  almost  reach  the 
postero-median  column.  (2)  The  fibres  that  enter  the  caput  directly, 
pass  through  this  to  the  posterior  horn,  some  on  their  level  of  entrance 
(and  these,  in  the  lumbar  region,  have  a  curved  course  like  the  meridian 
lines  on  a  globe),  while  others  turn  upward  in  compact  bundles  to 
pass  forward  into  the  cornu  at  a  higher  level.  (3)  The  fibres  of 
Lissauer's  group  also  enter  the  horn,  either  through  or  in  front  of  the 
caput,  after  a  short  ascending  coxirse. 

YOL.  I.  15 


226  SPINAL    CORD. 

Thiis,  except  the  fibres  to  the  poster o-median  column  (which  ascend 
in  it  without  decussating),  all  the  other  posterior  root-fibres  enter  the 
posterior  horn,  chiefly  on  its  inner  side  or  through  the  caput,  a  few 
(Lissauer's)  on  its  outer  side.* 

In  the  posterior  horn,  fibres  of  each  set  pass  to  the  posterior 
commissure ;  others  go  to  the  posterior  vesicular  column  (where  this 
exists),  while  some  pass  forwards  into  the  anterior  cornu,  chiefly 
towards  the  outer  group  of  nerve-cells ;  a  few  pass  towards  the  anterior 
commissure,  and  a  considerable  number  probably  end  in  the  nerve-cells 
of  the  horn.  The  fibres  that  pass  to  the  posterior  vesicular  column 
breat  up  into  branches  which  interlace  with  the  dendrites  of  its  cells. 
Many  of  those  that  pass  towards  the  posterior  commissure  are  probably 
also  interrupted  by  nerve-cells  in  the  posterior  cornu.  Thus  the  chief 
destination  of  the  fibres  that  do  not  enter  the  postero-median  column 
must  be  regarded  as  the  anterior  cornu  of  the  same  side  and  the 
opposite  half  of  the  cord ;  and  this  is  probably  true  also  of  the  numer- 
ous fibres  that  end  in  the  nerve-cells  of  the  cornu.  The  fibres  from 
the  vesicular  tract  to  the  direct  cerebellar  tract  perhaps  continue  the 
path  of  the  posterior  root-fibres. 

Blood-stjpplt  to  the  Spinal  Coed. — The  arrangement  of  the 
arteries  in  the  cord  is  not  a  matter,  at  present,  of  much  practical 
importance.  Nevertheless  it  is  one  on  which  some  detailed  facts  are 
necessary,  because  it  is  a  subject  to  which  it  is  most  desirable  close 
attention  should  be  given  on  account  of  the  probability  that  vascular 
lesions  are  more  frequent  than  our  present  knowledge  indicates,  and 
the  relation  of  lesions  to  arterial  regions  should  be  carefully  noted 
The  chief  general  fact  is  that  the  blood- supply  is  in  part  central,  in 
part  peripheral;  the  central  supply  is  derived  from  the  branches  in 
the  anterior  median  fissure,  and  embraces  the  grey  matter,  except  the 
posterior  horn ;  the  peripheral  supplies  the  posterior  horn  (except  the 
neck)  and  the  white  substance. 

The  arterial  blood  is  brought  to  the  cord  by  small  branches,  derived 
from  the  vertebrals,  intercostals,  and  other  arteries,  which  reach  the 
cord  by  the  anterior  and  posterior  roots.  The  anterior  pass  for  the 
most  part  inwards  to  the  anterior  median  fissure,  where  they  ar« 
connected  by  vertical  branches,  continuous  in  direction,  so  as  to  form 
an  anterior  spinal  artery.  From  this  a  series  of  branches  pass  back- 
wards in  the  anterior  median  fissure,  which  may  be  called  anterior 
median  arteries,  and  are  of  great  importance,  supplying  most  of  the 
grey  matter.  At  the  bottom  of  the  fissure  each  divides  into  two 
branches,  a  riglit  and  left  commissural  artery,  which  passes  outwards 

*  The  fibres  that  piss  to  the  postero-external  column  have  heen  termed  the  "median 
group,"  and  the  rest  of  the  root- fibres  the  "lateral  group,"  those  entering  the  caput 
being  distiiiijuished  as  an  "  internitdiate  group."  But  each  investigator  describes 
much  the  same  facts  in  a  different  method,  and  adopts  an  original  nomenclature, 
which  renders  it  better  at  present  to  keep  the  facts  more  prominent  in  the  mind 
than  the  names. 


STEUCTUUB. 


227 


and  backwards  through  the  commissure,  displacing  its  fibres  (and 
hence,  in  section,  the  commissure  often  appears  to  be  interrupted 
where  the  fibres  are  divided  obliquely).  At  the  end  of  the  commis- 
sure each  divides  into  an  anterior  central  artery,  which  supphes  most 
of  the  anterior  horn,  and  a  posterior  central,  which  supplies  the  inter- 
mediate grey  matter  and  the  neck  of  the  posterior  horn,  including  the 
region  of  the  posterior  vesicular  column.  Each  commissural  artery, 
moreover,  before  dividing,  gives  of£  a  branch  which  immediately 
bifurcates  into  an  up- 
ward and  downward  ves-  — ^  ^^- 
Bel,  each  continuous  with 
a  corresponding  branch 
from  the  next  commis- 
sural artery  above  and 
below — the  anastomotic 
artery.  This  effects  a 
vertical  continuity  of 
anastomoses  within  the 
cord,  like  that  of  the 
anterior  spinal  artery 
outside  the  cord. 

The  peripheral  arteries 
pass  inwards  from  the 
surface.  K  posterior  me- 
dian artery  courses  in  the 
median  septum,  giving 
branches  to  each  side, 
and  an  intermediate  septal 
aHery  passes  in  the  cor- 
responding septum  out- 
side the  post-median 
column.  Midway  be- 
tween this  and  the  root- 
furrow  a  branch  enters 
and  passes  through  the  postero-external  column  to  the  posterior  horn, 
which  it  enters  in  front  of  the  caput  and  chiefly  supplies — ^the 
posterior  cornual  artery ;  while  a  posterior  radicular  artery  passes  in  on 
the  inner  side  of  the  posterior  root,  and  is  distributed  to  this  and  the 
caput.  A  series  of  anterior  radicular  arteries  enter  with  the  anterior 
roots,  and  between  them  and  the  posterior  horn  are  anterior,  middle, 
and  posterior  lateral  arteries  in  corresponding  parts  of  the  lateral 
column.  The  branches  into  the  lateral  column  do  not  reach  the 
grey  substance ;  the  branches  in  the  grey  substance  extend  into  the 
inner  part  of  the  lateral  column. 

The  veins  of  the  cord  correspond  in  the  main  to  the  arteries,  but  a 
large  proportion  of  the  blood  passes  into  the  peripheral  system. 
Some,  especially  those  of  the  white  substance,  pass  outwards  along 


Fig-.  83. — Semi-diagrammatic  representation  of  th« 
arteries  of  the  spinal  cord;  as,  anterior  spinal. 
Central  arteries. — a  m,  Hnterior  median ;  c  a,  be- 
tween the  right  and  left  commissural  arteries j 
a,  anastomotic  artery,  divided  transversely,  tc 
which  a  branch  goes  from  the  commissural  artery, 
which  then  divides  into  a  c,  interior  cornual,  and 
m  c,  mid-cornual  arteries.  Peripheral  arteries. — 
p  m,  posterior  median ;  p  i,  post-intermediate ;  p  e, 
posterior  cornual;  p  r,  posterior  rndicular;  p  I, 
m  I,  a  I,  regions  of  the  posterior,  middle,  and 
anterior  lateral  branches;  a  r,  anterior  radicular. 
In  the  right  half  of  the  figure  the  more  deeply 
shaded  part  indicates  the  region  supplied  by  the 
central  arteries. 


228  SPINAL    COED. 

the  septa;  and  those  on  the  surface  of  the  sides  and  back  of  the 
cord  are  gathered  into  a  posterior  spinal  vein.  Others,  especially 
of  the  grey  matter,  pass  inwards  to  a  large  vertical  vein  that  lies 
in  the  grey  commissure,  a  little  distance  from  the  central  canal  on 
each  side  (v.  Fig.  80 ;  compare  also  Fig.  65),  from  which  branches  pass 
forwards  to  the  anterior  median  fissure,  and  through  this  to  an 
anterior  spinal  vein.  The  anterior  and  posterior  spinal  veins 
deliver  their  blood,  through  communicating  veins,  into  the  large 
venous  plexuses  that  lie  outside  the  dura  mater,  and  which  receive 
blood  also  from  the  bones,  and  from  the  structures  and  skin  behind 
the  spine.  But  the  veins  of  the  spinal  cord  cannot  be  injected  from 
these  plexuses,  not  because  there  are  valves  in  the  connecting  branches, 
but  apparently  because  they  form  so  trifling  a  proportion  of  the  total 
connections.  From  the  plexuses  blood  passes  to  the  various  vertebral, 
cervical,  and  intercostal  veins. 

Although  there  is  a  vertical  continuity  of  the  vessels  of  the  cord, 
it  is  probable  that  the  course  of  the  circulation  is,  in  the  main,  hori- 
zontal. From  the  very  tortuous  course  of  the  path  by  which  arterial 
blood  enters  the  cord,  it  is  evident  that  the  pressure  to  which  the 
arteries  of  the  cord  are  exposed  must  be  far  lower  than  that  in  the 
arteries  of  the  brain,  and  hence  they  are  far  less  liable  to  degeneration 
and  to  rupture.  On  the  other  hand,  the  conditions  that  prevent  an 
injection  of  the  plexus  of  veins  outside  the  dura  mater  from  passing 
into  the  veins  of  the  cord,  must  save  the  latter  from  the  extreme  over- 
distension to  which  they  would  otherwise  be  liable  when  there  is  a 
hindrance  to  the  return  of  blood  from  the  plexus. 


FUNCTIONS    OF  TEF   SPINAL    CORD. 

"We  are  now  in  a  position  to  consider  the  functions  of  the  spinal 
cord,  and  the  structures  by  which  they  are  subserved.  The  functions 
are  these :  (1)  The  spinal  cord  conducts  motor  nerve  impulses  from 
the  brain  and  sensory  impulses  to  it.  (2)  It  constitutes  a  series  of 
centres  for  reflex  action.  (3)  It  contains  certain  centres  that  ulti- 
mately govern  nervous  action  in  structures  under  the  immediate 
control  of  the  sympathetic  system,  the  bladder,  rectum,  blood-vessels, 
&c.  (4)  It  influences  the  nutrition  in  all  parts  to  which  its  nerves 
extend. 

MoTOE  Conduction. — The  chief  facts  regarding  the  physiology  of 
the  motor  path  have  been  necessarily  considered  in  the  account  of 
its  anatomy.  We  have  already  seen  that  the  path  of  motor  impidses 
is  through  the  pyramidal  fibres  (in  the  anterior  and  lateral  pyramidal 
tracts),  the  anterior  cornua,  and  the  anterior  nerve-roots.    The  gan- 


FUNCTIONS.  229 

glion-cells  form  part  of  the  path,  and  so  also  probably  does  that  part 
of  the  plexus  of  fibrillse  in  the  comu  wliich  intervenes  between  the 
pyramidal  fibres  and  the  ganglion-cells.  We  have  seen  that  the 
crossed  relation  of  the  cerebral  hemisphere  with  the  limbs  is  sub- 
served by  the  decussation  at  the  pyramids,  and  apparently  to  a 
slight  extent  above  the  chief  crossing,  since  some  degenerated  fibres 
have  been  seen  in  the  anterior  pyramid  opposite  to  the  lesion  of  the 
brain.  We  have  seen  also  that  we  are  still  ignorant  of  the  mode  of 
connection  of  the  motor  cells  with  the  cerebral  hemisphere  of  the 
same  side  in  the  case  of  the  muscles  that  habitually  act  with  their 
fellows  on  the  opposite.  These  can  be  acted  on  by  either  hemi- 
sphere in  degree  that  approaches  equality  in  proportion  as  they  act 
together,  and  is  almost  complete  in  muscles  such  as  the  intercostals, 
which  can  only  act  together.  It  is  great  in  the  case  of  all  the  trunk 
muscles  and  of  those  of  the  leg,  less  in  those  of  the  arm.  This  relation 
is  further  considered  in  the  account  of  hemiplegia  in  Vol.  II,  to  which 
it  specially  pertains.  The  variations  in  the  decussation  of  the  chief 
set  of  fibres  suggest  that  the  variations  in  observed  facts  bearing  on 
the  question  may  merely  represent  variations  that  exist  in  nature. 
The  connection  may  be  subserved  by  the  fibres  already  mentioned  that 
never  decussate,  but  pass  into  the  lateral  pyramidal  tract  of  the  same 
side. 

The  large  fibres  of  the  anterior  motor  nerve-roots  are  far  more 
numerous  than  the  fibres  of  the  pyramidal  tracts,  and  so  also  are  the 
<;ells  of  the  anterior  horn,  from  which  the  large  root-fibres  proceed. 
Nevertheless  it  is  probable  that  all  these  cells  and  root-fibres  can  be 
excited  through  these  tracts.  Hence  many  cells  must  be  related  to 
each  of  the  pyramidal  fibres.  A  complex  mechanism  is  no  doubt 
established  by  the  interlacement  of  the  terminal  fibrillse  of  the 
pyramidal  axons  with  the  dendrites  of  the  anterior  cornual  cells. 
This  mechanism  is  such  that  the  stimulation  of  certain  pyramidal 
fibres  excites  to  action  a  much  greater  number  of  nerve-cells,  so  con- 
nected and  arranged  as  to  produce,  through  the  related  nerve-rocts,  a 
complex  movement,  in  which  many  muscles  may  take  part.  The  nerve- 
ceUs  thus  connected  may  not  all  be  at  precisely  the  same  level,  and 
only  some  of  those  at  a  given  level  may  be  thus  associated.  The 
simpler  the  movements,  and  the  fewer  their  possible  variations,  the 
fewer  pyramidal  fibres  may  suffice  for  the  production  of  the  move- 
ments. Thus  it  is  conceivable  that  for  such  movements  as  those  of 
the  intercostal  muscles,  which  are  simple  and  constant,  very  few 
pyramidal  fibres  may  suffice,  whereas  a  much  larger  number  must  be 
necessary  for  the  highly  variable  movements  of  the  hand.  It  is  pro- 
bable also  that  the  pyramidal  impulses  influence  other  neurons  than 
those  directly  subserving  muscular  contraction.  By  means  of  such 
intermediate  or  associational  neurons  these  impulses  are  enabled  to 
effect  a  more  complex  and  varying  m.ovement  than  could  otherwise  be 
produced. 


230  SPINAL    CORD. 

Eepresbntation  of  Movemehtts  in  the  Spiital  Cord. — It 
important  to  inquire  how  far  we  can  identify  these  yarions  motor 
mechanisms  of  the  anterior  grey  matter.  There  are  several  sources  of 
information  on  this  subject.  We  have  already  seen  that  the  arrange- 
ment of  fibres  in  the  anterior  roots  is  such  as  to  associate  certain  move- 
ments with  certain  nerve-roots,  and  it  is  a  reasonable  assumption  that 
this  corresponds,  to  some  extent  at  least,  with  the  arrangement  in  the 
grey  matter.  The  sources  of  our  information  on  this  point  are  the 
limited  facts  of  anatomy,  rare  cases  of  restricted  disease  of  the  nerve- 
roots  in  man,  and  especially  the  experiments  of  Terrier  and  Teo  on 
monkeys.  We  have  already  considered  the  indications  that  these 
experiments  afford,  and  their  suggestiveness,  and  we  have  seen  that 
their  value  is  relative  rather  than  absolute.  As  regards  the  grey  matter, 
we  must  remember  also  that  there  may  not  be  a  strict  horizontal  corre- 
spondence between  the  nerve-roots  and  the  nerve-cells,  because  it  is 
probable  that  some  root-fibres  enter  the  antero-lateral  white  columns, 
and  are  connected  with  nerve-cells  at  a  higher  or  lower  level  than  that 
at  which  they  leave  the  cord.  Another  source  of  information  is  the 
degeneration  of  nerve- cells  that  follows  slowly  on  an  amputation  of  a 
limb,  and  the  condition  of  the  spinal  cord  in  congenital  absence  of 
part  of  a  limb.  The  last  and  most  important  source  of  information  is 
supplied  by  cases  of  limited  disease  of  the  anterior  cornua,  in  which 
the  position  of  the  lesion  and  the  distribution  of  the  resulting  palsy 
can  be  compared.  Destruction  of  nerve-cells  causes  degeneration  of 
the  motor  fibres  proceeding  from  them,  and  wasting  of  the  muscles  to 
which  those  motor  fibres  proceed.  This  alone  affords  us  certain  know- 
ledge. Cases  of  clear  significance  are,  however,  rare,  and  it  will  be 
long  before  our  knowledge  can  be  complete.  Meanwhile  we  may 
learn  something  of  the  central  association  of  muscles  by  observing 
what  muscles  are  most  freq[uently  paralysed  together  by  such  disease. 
This  subject  has  been  carefully  studied  by  E.  Eemak*  and  by 
Thorburn,t  and  many  valuable  isolated  observations  have  been  pub- 
hshed.  It  is  only  the  associations  which  are  frequent  that  can  be 
allowed  significance,  because  it  is  not  uncommon  to  have  more  than 
one  focus  of  disease  in  the  grey  matter.  The  following  summary 
presents  the  conclusions  that  seem  probable  from  the  evidence  at 
present  available. ;{: 

The  various  facts  for  the  most  part  fully  confirm  the  conclusion  of 

*  *  Arcliiv  f.  Psychiatrie,*  vol.  ix. 

t  'Brain,'  January  7th,  1887,  and  October,  1888.  The  facts  were  observed  on 
cases  of  injury.  He  associates  each  muscle  with  a  single  spinal  nerve,  but  the^e 
nerves  are  only  collections  of  roots  that  are  severally  continuous,  as  are  the  groups 
of  nerve-cells  within  the  cord,  and  it  is  certain  that  the  roots  related  to  many  of 
the  muscles  go  into  adjacent  spinal  nerves. 

J  A  useful  summary  of  the  evidenoe  up  to  the  middle  of  1884  was  given  by  Dr. 
Allen  Starr,  in  the  'American  Journal  of  Neurology,'  Aug.  and  Nov.,  1884.  The 
subject  is  a  tempting  one  for  theory,  but  it  is  very  important  to  keep  to  the  solid 
ground  of  facts,  however  limited  it  may  be  in  extent. 


FUNCTIONS.  231 

fJemalr,  corroborated  by  tbe  experiments  of  Perrier  and  Teo,  that 
most  movements  and  muscles  are  represented  in  vertical  tracts,  and  the 
whole  anterior  grey  matter,  at  any  one  nerve-segment,  contains  cells 
that  are  concerned  with  different  movements.  An  extensive  lesion  of 
small  vertical  extent  may  thus  weaken  many  movements,  but  abolish 
none.  The  special  representation  of  the  muscular  functions,  i.e.  of 
definite  movements,  is  no  doubt  related  to  special  groups  of  nerve- 
cells,  but  a  single  group  may  be  concerned  in  more  than  one  associated 
movement.  Different  groups  are  probably  intimately  connected, 
because  we  kaow  that  the  contraction  of  any  muscle  is  accompanied 
by  a  slighter  but  proportioned  contraction  of  its  antagonists.  Anato- 
mical connections  in  both  spinal  cord  and  brain  doubtless  underlie  this 
association. 

Cervical  Region. — The  highest  cervical  region  apparently  contains  centres 
for  the  small  rotators  of  the  head,  and  the  depressors  of  the  hyoid  bone  were 
found  by  Beevor  and  Horsley  to  be  related  to  the  first  two  pairs  of  nerves,  the 
sterno-hyoid  and  sterno-thyroid  chiefly  to  the  first  and  the  omohyoid  to  the 
second.*  The  upper  part  also  contains  centres  for  the  muscles  of  the  neck, 
especially  for  the  sterno-mastoid  and  upper  part  of  the  trapezius.  The  dia- 
phragm is  probably  represented  in  the  grey  matter  at  the  level  of  the  roots  of 
the  phrenic  nerve,  the  fourth  cervical.  In  the  brachial  reo:ion  of  the  cervical 
enlargement,  the  muscles  of  the  shoulder  are  represented  chiefly  in  the  upper 
part;  the  intrinsic  muscles  of  the  hand  in  the  lower  part.  The  flexors  of  the 
elbow  ai'e  represented  above  its  extensors,  and  the  supinators  and  extensors 
of  the  wrist  above  the  flexors  of  the  wrist. 

Deltoid,  scapular  muscles,  pectoralis,  and  serratus. — The  fifth  and  sixth 
segments,  for  the  most  part  the  sixth,  and  probably  the  outer  group  of  nerve- 
cells.f  The  centres  for  the  two  parts  of  the  pectoralis  are  separate,  and  are 
associated — the  clavicular  with  that  of  the  serratus  magnus,  the  costal  with 
that  of  the  latissimus  dorsi  (see  p.  37).  These  associated  muscles  are  repre- 
sented near  together,  but  not  at  the  same  spot ;  the  association  is  often 
reproduced  in  disease  of  the  anterior  cornu,  the  clavicular  part  being  affected 
together  with  the  serratus,  but  one  muscle  may  suffer  without  that  which  is 
commonly  associated  with  it. 

The  Flexors  of  the  elbow  and  supinators  probably  correspond  nearly  in  level 
with  the  deltoid.  The  whole  of  this  series  of  muscles  may  be  affected  alone  by 
disease  of  the  nerve-roots  (see  p.  38)  or  of  the  grey  matter,  and  then  we  have 
the  "  upper  arm  type  "  of  palsy,  described  first  by  Erb. 

Extensor  of  the  elbow  (triceps)  :  probably  the  middle  of  the  brachial  region, 
chiefly  the  seventh  segment,  extending  down  to  the  eighth,  and  perhaps  up  to 
the  sixth,  possibly  chiefly  in  the  outer  group  of  nerve-cells. 

The  Extensors  of  the  wrist  are  represented  above  those  of  the  elbow  ; 
probably  chiefly  at  the  sixth  or  sixth  and  seventh  segments,  and  not  from  the 

*  Chiefly  the  posterior  belly  of  the  muscle,  by  a  branch  joining  the  glosso- 
pharyugeal.  The  other  muscles  named  (including  slightly  the  anterior  belly  of  the 
omohyoid)  are  innervated  from  the  first  cervical  by  a  branch  joining  the  hypoglossal. 

t  Thorburn  (loc.  cit.)  refers  the  infra-  and  supra-spinati  with  much  probability 
to  the  fourth  pair  of  nerves. 


232  SPINAL   CORD, 

pnstero-external,  l)ut  from  the  other  groups.  Flexors  of  the  tcrist :  below  tha 
extensoi-s,  nearly  on  the  same  level  as  the  extensors  of  the  elbow,  at  the  seventb 
and  eighth  segments,  and  probably  also  from  one  of  the  outer  groiips  of  cells. 
The  pronators  have  nearly  the  same  representation  as  the  flexors  of  the  wrist. 

Long  Extensors  of  the  fingers  :  upper  part  of  the  brachial  region,  about  the 
level  of  tiie  sixth  or  between  it  and  the  seventh  segment;  prol)a"hly  from  one  of 
the  anterior  groups  (not  from  the  postero-lateral  group).  Long  flexors  of  the 
fingers:  below  the  extensors;  probably  seventh  or  eighth  segment. 

Intrinsic  muscles  of  the  hand:  lowest  part  of  the  brachial  region,  and  the 
thenar  muscles  a  little  higher  than  the  interossei ;  the  latter  being  represented 
in  the  first  dorsal  segment.*  The  intrinsic  muscles  (like  the  long  extensors) 
are  probably  related  less  to  the  outer  than  to  the  inner  and  anterior  groups. 
The  two  sets  of  muscles  are  related  both  in  action  and  in  central  connection,  as 
was  illusti-ated  by  a  case  of  concussion-lesion  under  my  care,  in  which  the  two 
outer  interossei  were  paralysed  and  wasted,  and  also  the  part  of  the  long 
extensor  supplying  the  two  outer  fingers,  but  no  other  muscles. 

Lumbar  Enlaroement- — Our  knowledge  of  the  representation  of  muscles  in 
the  lumbar  enlargement  is  very  slight,  and  we  have  scarcely  any  definite  facts 
concerning  their  relation  to  the  cell-groups.  The  postero-external  group  pre- 
ponderates over  the  others  in  size,  even  more  than  in  the  cervical  cord,  and 
doubtless  also  in  importance.  The  few  pathological  observations  of  changes  in 
the  several  groups  are  not  altogether  consistent.  The  chief  facts  available 
relate  to  the  probable  segmental  level  and  association  of  the  centres,  and  are  as 
follows : 

Cremaster,  second  lumbar  segment.  Psoas,  second,  iliacus,  third  lumbar; 
the  two  muscles  (one  in  function)  are  probably  related  to  a  group  of  cells  ex- 
tending through  both  segments.  Adductors,  fourth  lumbar  segment.  Gluteal 
muscles  (extensors  of  hip),  fourth  and  fifth  lumbar  segments. 

Extensors  of  Ttnee,  third  and  fourth  lumbar  segments,  probably  from  the 
same  group  as  the  flexors  of  the  hip  ;  the  two  sets  of  muscles  are  often  affected 
together,  and  sometimes  the  abductors  suffer  with  them.  The  Sartorius  is 
probably  related  to  the  third  segment,  but  not  to  the  same  cell-group  as  the 
extensors  of  the  knee  (to  which,  indeed,  it  does  not  belong;  see  p.  47).  It 
usually  escapes  in  atrophic  palsy  of  the  extensors.  Flexors  ofhnee,  fifth  lumbar 
and  first  sacral  segments.  Their  centre  is  thus  below  and  certainly  distinct 
from  that  of  the  extensors. 

The  muscles  of  the  lower  leg,  moving  the  foot  and  toes,  are  related  to  the 
fourth  anJ  fifth  lumbar  and  the  first  sacral  segments.  The  calf  muscles,  the 
tibialis  anticus,  and  the  peroneus  longus  have  certainly  sejiarate  centres,  and 
ai-e  often  affected  separately.  The  tibialis  anticus  may  suffer  alone,  or  may 
alone  escape  when  the  other  muscles  in  front  of  the  leg  are  paralysed.  The 
muscles  of  the  calf  may  be  affected  when  those  in  front  of  the  leg  are  not.  It 
is  probable  that  the  peroneus  longus  is  the  highest  in  central  representation, 
and  is  related  to  the  fourth  lumbar  segment. 

The  intrinsic  muscles  of  the  foot,  especially  the  interossei,  are  the  lowest  in 

•  This  is  the  indication  of  the  majority  of  cases.  Nevertheless  Sahli  has  recorded 
a  case  of  atrophy  of  all  the  intrinsic  muscles  of  the  hand,  in  which  the  lesion  did 
not  extend  below  the  seventh  segment ;  the  eighth  and  first  dorsal  were  perfectly 
niirmal.  I  have  seen  a  case  of  atrophy  limited  to  the  deltoid  and  intrinsic  muscles 
of  the  hand,  due  to  an  acute  process,  probably  hsemorrhage,  but  in  this  case  there 
may  have  been  two  lesions. 


Ji'DNOTIONS.  233 

central  relations,  being  connected  with  the  second  sacral  segment.  The  position 
of  their  centres,  in  the  lowest  part  of  the  lumbar  enlargement,  thus  corresponds 
to  that  of  the  analogous  muscles  o£  the  hand,  in  the  lowest  part  of  the  cervical 
enlargement. 

Sensoet  CoNDxrcTioiT. — Our  knowledge  of  tlie  sensory  path,  in  the 
cord  is  far  less  definite  than  that  of  the  motor  path,  in  spite  of  the 
fact  that  it  has  been  the  subject  of  a  large  number  of  experimental 
investigations.  The  subject  is  a  very  difficult  one  for  experimental 
solution,  on  account  of  the  difficulty  of  ascertaining  the  condition  of 
sensation  in  animals.  The  indications  afforded  by  disease  are  equivocal 
for  another  reason.  We  have  seen  (p.  78)  that,  in  the  sensory  nerves, 
a  lesion  which  permanently  interrupts  motor  conduction  may  scarcely 
affect  sensory  conduction,  and  the  same  thing  is  apparently  true  of 
the  spinal  cord.  If  loss  of  feeling  results  from  an  acute  lesion,  it 
often  q"iickly  passes  away,  although  motor  palsy  remains  complete 
and  absolute,  and  this  when  all  the  elements  of  the  cord  seem  equally 
implicated  in  the  morbid  process.  In  cases  of  such  chronic  disease 
as  compression,  which  when  slow  must  act  on  all  the  structures  in 
nearly  equal  degree,  the  same  escape  of  sensory  conduction  is  often 
observed.  Difficulties  are  encountered  whatever  mode  of  interpretation 
of  the  phenomenon  we  adopt,  and  the  fact  of  importance  to  our  present 
problem  is  that  we  are  not  yet  able  strictly  to  compare  structural  and 
functional  damage  when  the  former  is  moderate.  When  the  damage  has 
been  slowly  produced  it  may  be  apparently  great,  and  yet  function  may 
not  be  much  impaired,  especially  the  lower  function  of  conduction  of 
nerve-force.  The  significance  of  these  facts  as  regards  the  problem  of 
sensory  conduction  in  the  cord  is  that  we  must  be  cautious  in  drawing 
a.ny  conclusion  from  the  persistence  of  sensation.  We  cannot  infer, 
because  supposed  sensory  tracts  are  visibly  diseased  in  a  case  in  which 
there  was  no  loss  of  sensibility,  that  these  tracts  have  not  the  function 
assigned  to  them,  unless  we  can  feel  sure  that  all  the  axis-cylinders 
are  destroyed.  This  we  can  seldom  do;  and  for  this  reason,  and 
because  morbid  processes  are  often  wide  in  distribution,  the  evidence 
that  can  be  obtained  by  comparing  the  position  of  lesions  with  the 
sensory  symptoms  they  produce  is  limited  and  uncertain.  Other 
sources  of  information  are  the  effect  of  partial  lesions  of  the  cord, 
intense  in  degree,  but  limited  in  area,  especially  traumatic  lesions 
affecting  one  half  of  the  cord  or  part  of  one  half.  Cases  of  this  kind 
are  of  high  value,  but  in  few,  at  present,  have  the  extent  of  lesion  and 
the  range  of  symptoms  been  accurately  ascertained. 

Hence  physiologists  have  relied  chiefly  on  the  results  of  experiments  on 
animals.  Unfortunately  the  help  these  can  give  is  also  reduced  to  a  literal 
minimum  by  the  difficulties  of  experimentation  and  of  interpretation — the 
physical  difficulties  on  the  one  hand,  the  functional  perplexities  on  the  other. 
Some  clear  facts  have  been  ascertained,  and  other  results  have  been  obtained 
that  still  wait  certain  interpretation.  It  seems  certain,  in  the  first  place,  that 
sensation  in  any  particular  region  of  the  skin  is  associated  with  the  functional 


234  SPINAL    CORD. 

activity  of  a  definite  portion  of  the  opposite  cerebral  cortex.  Anatomical 
evidence  shows  that  the  axons  belonging  to  different  centripetal  tracts  cross 
the  middle  line  at  various  levels  in  the  cord,  medulla,  and  mid-brain.  The 
eai-ly  experiments  of  Brown-Sequard  led  him  to  conclude  that  the  chief  part  of 
the  sensory  path  decussates  soon  after  entering  the  cord,  and  with  this  view 
pathological  facts  in  general  agree.  The  consensus  of  evidence,  however, 
points  to  a  less  sudden  and  uniform  crossing  than  was  formerly  assumed.  The 
axons  entering  from  the  periphery  do  not  as  a  rule  pass  directly  across  the  cord, 
but  break  up  in  the  grey  matter,  whence,  possibly  after  one  or  more  relays,  the 
conducting  path  is  continued  on  the  opposite  side.  It  will  be  seen  that  these 
considerations  suggest  that  sensory  conduction  is  a  function  of  the  lateral  and 
anterolateral  tracts  rather  than  of  the  posterior  columns,  which  run  uncrossed 
to  the  region  of  the  medulla.  This  is  quite  in  accordance  with  what  is  found 
both  clinically  and  experimentally.  For  the  posterior  median  fibres  may  be 
extensively  degenerated  when  sensation  is  unimpaired,  a  fact  of  which  we  shall 
see  the  probable  meaning  presently;  and,  on  the  other  hand,  Osawa  has  shown 
that  section  across  the  whole  cord,  with  the  exception  of  the  posterior  column, 
completely  abolishes  sensation.  That  this  abolition  is  incomplete  unless  the 
grey  matter  is  completelv  divided  was  found  also  by  SchifE ;  the  important 
part  played  by  the  grey  matter  in  sensoiy  conduction  is  thus  confirmed. 

The  subject,  however,  at  the  present  time  is  too  far  from  even  the  semblance 
of  settlement  to  make  a  discussion  of  the  evidence  worth  the  space  it  would 
need,  especially  in  a  work  dealing  with  practical  medicine.*  It  is  only  desir- 
able,  therefore,  to  point  out  certain  leading  facts  and  leading  considerations. 

Clinical  evidence,  largely  based  upon  cases  of  syringomyelia,  points  to  the 
grey  matter  as  the  seat  of  conduction  of  painful  impressions.  Experiments 
suggest  that  impressions  which  act  upon  the  vaso-motor  centre  pass  up  in  the 
lateral  columns — in  animals  chiefly  on  the  side  of  the  cord  opposite  to  that 
on  which  they  are  produced,  slightly  on  the  same  side.  Sensations  of  touch 
appear  to  be  conducted  mainly  in  the  lateral  columns  and  grey  matter.  In 
man  the  paths  for  both  touch  and  pain  are  on  the  opposite  side  of  the  cord. 

We  may  accept  as  certain  the  fact  that  the  tactile  or  painful  impulse  we  feel 
crosses  the  middle  line  soon  after  entering  the  cord;  it  is  probable  that  some 
impulses  that  we  do  not  feel  do  not  cross.  We  must  never  forget  that  there  is 
strong  reason  to  believe  that  only  a  minority  of  the  impulses  that  tiu verse 
afferent  nerves  affect  our  consciousness. 

The  participation  of  the  grey  matter  in  the  conduction  of  afferent  impressions 
has  been  fully  confirmed  by  the  facts  as  to  the  course  of  centripetal  fibres  in  the 
cord  revealed  by  the  recent  improvements  in  histological  technique.  That 
sensation  escapes  in  anterior  polio-myelitis  points  to  the  posterior  part  of  the 
o-rey  matter  as  the  seat  of  this  conduction,  and  this  again  is  in  agreement  with 
both  histological  and  experimentalf  evidence. 

The  assumT)tion  that  a  certain  number  of  centripetal  axons  exist  in  the  central 
grey  matte?  is  of  great  service  in  studying  the  problems  of  sensory  conduction. 

Still,  fhoy  are  not  numerous  enough  to  save  us  from  the  difficulty  that  the 


*  Thus,  for  exiiniple,  two  of  the  latest;  series  of  experiiiieuts  upon  monkeys,  those 
of  Mott  and  Turner,  yield  conflicting  results.  The  latter  are  mainly  confirmatory 
of  t'lose  ot  Brown-Sequard ;  while,  according'  to  the  lormer,  touch  and  the  muscular 
s»'  se  are  mainly  transmitted  up  along  the  side  of  the  cord  at  which  they  enter,  but 

e  paths  of  pain  and  temperature  pass  up  on  both  sides. 

t  E.  g.  tlie  researches  of  Schiff  and  Ciaglinski  (see  later). 


FUNCTIONS.  235 

fibres  we  can  look  to  are,  taken  altogether,  insufficient  in  number  to  subserve 
the  marvellous  difEerentiation  of  sensation  in  the  periphery — differentiation  in 
quality  of  feeling  and  especially  in  locality  of  feeling.  But  a  difficulty  may 
become  inoperative,  without  ceasing  to  exist,  by  being  absorbed  in  a  still  larger 
difficulty  of  the  same  kind.  If  we  avail  ourselves  of  all  the  structures  of  the 
spinal  cord  that  can  conceivably  subserve  upward  conduction,  we  fail  to  find 
room  for  the  separate  conduction  of  impulses  which,  nevertheless,  the  spinal 
cord  must  separately  convey. 

On  only  one  hypothesis  is  if  possible  to  conceive  such  conduction.  It  has 
already  been  pointed  out  that  the  increased  power  of  the  microscope  has  shown 
us  that  each  axis- cylinder,  so  called,  is  not  a  simple  body,  but  a  compound 
structure,  composed  of  fibrillae,  and  comparable,  in  point  of  fact,  to  a  bundle  of 
nerve-fibres.  What  was  formerly  regarded  as  division  of  an  axis-cylinder  is 
now  known  to  be  the  separation  of  its  ultimate  fibrillae,  and  this  has  been 
shown  to  occur  in  both  motor  and  sensory  axons,  and,  to  a  certain  extent,  along 
their  course  (collaterally)  as  well  as  at  their  terminations.  It  seems  to  follow 
of  necessity  that  feach  sensory  area  of  distinct  perception  must  be  subserved  by 
one  of  these  fibrillae.  It  is  conceivable  that  the  impulses  may  keep  each  to  its 
own  fibrillary  path,  however  many  fibrillae  an  axis-cylinder  may  contain.  We 
have,  indeed,  seen  that  such  fibrillae  keep  separate  even  in  traversing  a  nerve- 
cell.  It  is  only  by  such  an  arrangement  that  it  seems  possible  for  the  sensory 
impressions  from  skin  and  tissue,  of  touch,  pain,  and  temperature,  to  be  carried 
to  the  brain  so  as  separately  to  excite  certain  nerve-cells  of  the  cortex. 

If  these  considerations  have  weight  we  cannot  exclude  the  lateral 
and  posterior  columns  from  the  function  of  sensory  conduction  on 
account  of  the  scanty  fibres  we  can  discern  to  subserve  the  function, 
and  may  consider  what  evidence  on  the  point  disease  and  injury 
afford  in  man. 

Unilateral  lesions  of  the  spinal  cord,  which  cause  loss  of  sensibility 
of  the  skin,  do  so  on  the  side  opposite  to  the  lesion.  This  is  true  of 
all  forms  of  sensibility.  They  thus  prove,  beyond  doubt,  the  de- 
cussation in  the  spinal  cord  of  the  path  from  the  cutaneous  nerves 
of  pain,  of  touch,  and  of  temperature.  An  analysis  of  the  cases  in 
which  both  effect  and  lesion  have  been  exactly  ascertained — which 
will  be  considered  more  fully  when  these  lesions  are  described — sug- 
gests certain  other  conclusions  regarding  the  position  of  these  paths. 
The  facts  suggest,  first,  that  the  paths  for  sensations  of  pain  and 
of  touch  are  not  near  together.  Sensibility  to  pain  has  been  lost  in 
almost  all  recorded  cases,  but  that  to  touch  in  only  two  thirds. 
They  suggest,  secondly,  that  the  two  paths  for  tactile  sensibility 
are  nearer  together  than  are  the  two  paths  for  pain.  In  no  case 
of  chiefly  unilateral  lesion  has  sensibility  to  pain  been  lost  on  both 
sides,  whereas  in  two  recorded  cases  sensib'lity  to  pain  was  lost  on  the 
side  opposite  to  the  lesion,  while  that  to  touch  was  lost  on  both  sides.* 

*  It  is  possible  tbiit  tactile  sensibility  may  have  been  lost,  in  other  cases,  on  the 
second  side,  t.  e.  on  the  side  of  the  lesion,  and  the  loss  may  have  escaped  notice  in 
consequence  of  the  exaltation  of  sensibility  which  is  commonly  present,  and  which 
is  presumably  due  to  the  nerves  of  common  sensibility. 


236  SPINAL    CORD. 

Ttese  conclusions,  moreover,  are  supported  by  the  facts  of  two  impor. 
tant  cases  of  injuiy  to  the  cord  which  afford  direct  localisiug  indica- 
tions. One  is  a  case,  recorded  by  myself,*  in  which  the  spinal  cord  in 
the  upper  cervical  region  was  damaged  by  a  small  spiculum  of  bone 
being  driven  against  it  by  a  bullet,  which,  entering  the  mouth,  lodged 
in  the  body  of  the  third  cervical  vertebra.  The  extent  of  the  lesion  of 
the  cord  is  shown  in  Pig.  84.     The  chief  injury  is  clearly  to  the  lateral 

column  and  grey  matter,  the  posterior 
column  being  merely  swollen,  appa- 
rently by  oedema.  The  affected  part 
was  the  seat  of  haemorrhage  and 
hcomorrhagic  infiltration.  The  effect 
was  entire  loss  of  sensibility  to  pain 
on  the  opposite  side  without  any  im- 
pairment of  tactile  sensibility.  To 
this    the    other     case,    recorded    by 

„  Q.  o  .-  i.  .  1  J  ,  Miiller,t  is  almost  a  complement.  A 
t  T».  o4. — Section  of  spinal  cord  be-  ,  tt-ttj^i  ii 

fcween  the  second  and  third  cervical  stab- wound  divided  the  whole  of  one 
nerves,  contused  by  gunshot  injury,  half  of  the  cord,  including  the  poste- 
rior column,  and  also  the  posterior 
column  of  the  other  side  almost  up  to  the  nerve-roots;  the  grey 
matter  of  this  side  escaped.  The  whole  of  both  postero-meiian 
columns  was  thus  divided.  The  effect  was  loss  of  sensibility  to  pain 
on  the  side  opposite  to  the  lesion,  and  of  that  to  touch  on  both  sides. 
Thus  there  is  strong  reason  to  believe  that 'the  path  for  sensibility 
to  pain  does  not  pass  in  the  posterior  columns.  The  evidence  of 
disease  (syringomyelia)  points  to  the  grey  matter  as  at  any  rate  the 
main  seat  of  this  path.  We  have  already  seen  that  the  escape  of 
sensation  in  anterior  polio-myelitis  indicates  that  it  must  pass  along 
the  posterior  part  of  the  grey  matter,  and  this  accords  well  with 
experimental  evidence.  Thus  Schiff  found  that  section  through  the 
whole  cord  except  the  posterior  columns  abolished  sensation  to  pain 
below  the  lesion,  whereas  i£  a  trace  of  the  posterior  part  of  the  grey 
matter  was  left  this  sensation  was  still  transmitted,  though  delayed. 
Ciaglinski  J  describes  an  ascending  tract,  found  experimentally  in  the 
dog  and  verified  histologically,  in  man,  between  the  postero- median 
column  and  the  central  canal,  and  regards  this  as  the  path  for  impres- 
sions of  pain  and  temperature.  As  to  the  path  of  tactile  sensibility 
we  must  wait  for  further  evidence.  The  fibres  that  degeneiate 
upwards  in  the  median  columns,  passing  to  them  from  the  external 
posterior  columns,  and   constituting  so  large  a  proportion  of  their 

*  '  Clinical  Society's  Trans.,*  vol.  xi,  1878,  p.  24. 

t  *  Beitrage  zur  path.  Anat.  und  Phys.  der  Ruckenm.,*  Leipzig,  1871.  Abstracts, 
which  suppleuienc  each  other,  and,  together,  give  a  full  iiccount  of  the  case,  will  be 
found  in  Kobuer's  article  on  unilateial  lesions,  'Dent.  Arch.  f.  klin.  Med.,'  1877| 
Bd.  xix,  p.  190 ;  and  in  '  Virchow's  Jahresbericht,'  1871,  Bd.  i,  p.  152. 

X  Cia^'linski, '  Neurol.  Centralbl.,'  1896. 


FUNCTIONS,  237 

mass,  apparently  come  from  tlie  muscles.  We  may,  however,  re- 
member that  many  fibres  pass  to  the  posterior  columns  from  the 
grey  matter,  and  also  from  the  posterior  commissure  in  the  middle 
line ;  some  of  the  former  may  also  come  from  the  posterior  commis- 
sure, and  both  these  sets  of  fibres  may  have  crossed  in  the  middle  line. 
It  is  possible  that  these  fibres  conduct  tactile  sensibility,  and  that 
they  are  mingled  with  those  that  conduct  muscular  sensibility.  This 
theory,  however,  can  only  be  regarded  as  a  suggestion  for  future 
observations;  it  must  be  confessed  that  the  results  of  experiment, 
which  are,  however,  for  reasons  already  stated,  not  so  vahd  as  clinical 
observations,  tend  to  indicate  the  implication  of  the  lateral  and  antero- 
lateral columns  in  the  conduction  of  tactile  sensations. 

The  conduction  of  impulses  from  the  muscles  is  certainly  in  the 
posterior  median  columns.  This  is  clearly  shown  by  the  facts  of  the 
pathology  of  tabes.  The  function  of  the  direct  cerebellar  tract  has 
still  to  be  demonstrated.  There  are  certain  resemblances  between  its 
fibre-relations  and  those  of  the  posterior  median  columns  which  give 
countenance  to  Flechsig's  theory  that  it  conveys  impressions  from  the 
muscles  of  the  lower  part  of  the  trunk  and  between  the  trunk  and 
lower  limbs.  But  the  origia  of  its  fibres  from  the  cells  of  the  poste- 
rior vesicular  column,  and  the  mystery  attaching  to  the  latter,  give 
additional  obscurity  to  this  structure.  The  fine  nerve-plexus  between 
the  cells  seems  connected  with  fibres  of  the  posterior  roots,  and  this 
plexus  is  said  to  atrophy  early  in  locomotor  ataxy.*  The  obscurity 
will,  indeed,  be  dispelled  if  we  can  accept  the  speculation  of  Sher- 
rington that  this  cylinder  is  really  part  of  the  series  of  ganglia  on  the 
posterior  nerve-roots,  which  occupies  a  position  within  the  spinal  cord, 
and  is  continuous  instead  of  broken  into  ganglia. 

The  path  for  sensations  of  temperature  is  still  unknown.  The  fact 
that  this  sense  is  frequently  impaired  with  sensibility  to  pain  suggests 
that  the  two  paths  may  be  near  together  in  the  grey  matter.  More 
than  this  cannot  be  said. 

But  we  have  to  consider  another  problem  in  our  study  of  the 
sensory  path.  The  same  impression  that  is  felt  may  excite  a  reflex 
action.  For  this,  afferent  root-fibres  must  end  in  the  grey  matter  of 
the  cord.  Are  the  two  functions  subserved  by  the  same  or  by  different 
fibres  ?  A  similar  question  presents  itself  with  regard  to  the  muscle 
nerves.  K  the  ascending  degeneration  in  the  postero-median  column, 
when  the  cauda  equina  is  diseased,  is  admitted,  as  it  must  be,  as 
proof  that  these  root-fibres  pass  up  without  interruption,  the  muscle- 
reflex  action  must  be  subserved  by  other  fibres.  For  it,  however, 
comparatively  few  may  suffice.  With  regard  to  the  other  sensory 
fibres,  the  evidence  at  present  suggests  that  all  undergo  interruption 
in  the  spinal  cord,  since  no  other  paths  have  yet  been  found  degene- 
rated when  a  lesion  has  been  confined  to  the  nerve-roots.     If  so,  the 

*  Lissauer,  Oppenheim,  &c.  See  Mott,  "Bipolar  Cells  of  the  Spinal  Co  d," 
'  Biiiin,'  part  lii,  1890. 


238  SPINAL    CORD. 

same  fibres  probably  subserve  sensation  and  reflex  action,  the  den- 
drites of  the  nerve-cells  providing  a  mechanism  for  the  division  of  the 
path  and  diversion  of  the  impulses.  No  impression,  in  health,  causes 
a  true  reflex  action  that  does  not  also  produce  a  conscious  sensation. 

Our  knowledge  of  the  serial  representation  of  cutaneous  sensibihty  in 
the  spinal  cord  has  of  late  been  much  increased  by  the  careful  researches 
of  Head,  Thorbum,  Starr,  Dana,  and  others.  It  is  probable  that  there 
is  a  progressive  i-epresentation  of  the  skin  in  the  posterior  nerve-ceUs 
"without  relation  to  the  nerve-trunks,  and  with  only  a  slight  relation  to 
the  nerve-roots.  Correspondence  with  the  latter  must  not  be  assumed. 
In  the  roots  the  serial  representation  is  along  the  axis  of  the  limb,  not 
across  it,  as  it  probably  is  in  the  nerve-cells.  In  the  dorsal  cord, 
ascending  disease  often  causes  an  affection  of  sensation  which  extends 
around  the  trunk  at  higher  and  higher  levels.  In  the  limbs  we  have 
indications  of  the  same  arrangement :  the  soles  and  palms  may  be 
affected  alone,  and  anaesthesia  may  extend  on  the  limb  to  a  certain 
level,  irrespective  of  nerve  distribution.  This  is  often  seen  in  the  legs. 
I  liave  known  anaesthesia  in  the  arms,  due  to  disease  of  the  cord,  to 
cease  midway  between  shoulder  and  elbow,  at  the  same  level  around 
each  arm.  Disease  of  the  cord  may  affect  sensation  on  the  tips  of  the 
fingers  only.  It  is  probable  that  the  ascending  course  of  many  of  the 
root-fibres  subserves  a  rearrangement  of  representation  in  the  cells, 
and  provides  also  for  a  special  connection  of  the  tactile  nerves  from 
each  part  of  the  skin  with  the  motor  cells  for  the  muscles  beneath — 
so  conspicuous  in  many  of  the  cutaneous  reflexes. 

Reflex  Action. — The  grey  matter  of  the  spinal  cord  constitutes  a 
series  of  reflex  centres,  some  of  which  must  be  of  considerable  ver- 
tical extent  and  much  complexity.  They  are  subserved  not  only  by  the 
grey  matter,  but  by  the  short  fibres  that  in  the  several  white  columns 
connect  the  grey  matter  at  different  levels.  The  passage  of  the  root- 
fibres  upwards  and  downwards  in  the  cord,  before  they  turn  into  the 
grey  matter  (conspicuous  in  the  posterior  roots),  also,  as  we  have 
noted,  constitutes  an  arrangement  for  spreading  the  afferent  impulses 
through  a  considerable  vertical  extent  of  the  cord. 

We  have  already  seen  (p.  19)  that  we  must  distinguish  two  forms 
of  reflex  action — the  cutaneous  reflex  action  and  the  muscle-reflex 
action,  the  latter  producing  the  "  myotatic  irritability  "  which,  as  we 
assume,  determines  the  so-called  "  tendon-reflex  contractions."  The 
first  form  of  reflex  action  is  not,  however,  limited  to  impressions  on 
the  cutaneous  nerves.  It  may  be  produced  by  stimulation  of  the 
nerves  supplying  the  deeper  structures,  including  those  of  the  tendons 
and  muscles.  The  attempt  to  obtain  the  knee-jerk  may  cause  a  true 
reflex  action  as  well  as  its  special  effect,  the  two  being  separated  by 
an  appreciable  interval  of  time  (see  Locomotor  Ataxy).  Eeflex  action 
from  the  skin  is  apparently  subserved  by  all  the  sensory  nerves, 
since  it  may  continue,  although  the  nerves  for  either  pain  or  touch 
have  ceased  to  act,  in  consequence  of  those  degenerative  processes  by 


FUNCTIONS.  239 

whicli  function  is  sometimes  abolisTied  in  as  complete  degree  and  with 
as  precise  limitation  as  the  physiological  inqtdrer  could  desire. 

The  central  process  concerned  in  reflex  action  must  take  place 
between  the  posterior  nerve-roots,  by  which  the  sensory  impulses 
reach  the  cord,  and  the  anterior  roots,  by  which  the  motor  impulses 
leave  it.  The  motor  ganglion-cells  form  part  of  the  reflex  centre,  and 
the  rest  of  the  centre  is  doubtless  constituted  by  the  fibrillary  plexus 
formed  by  the  inosculation  of  their  dendrites  and  those  of  the  sensory 
nerve-cells.  The  whole  path  between  and  including  the  in-  and  out- 
beariag  nerves  may  be  spoken  of  as  the  "  reflex  arc ;"  the  grey  matter 
concerned  being  called  the  "reflex  centre."  In  this  "centre"  the 
sensory  impulse  is  transformed  into — or  rather  gives  rise  to — a  motor 
impulse,  usually  much  greater  in  the  degree  of  its  energy.  Within 
the  centre  there  are  paths  established  by  the  connections  between  the 
nerve  structures,  of  which  only  a  few  are  in  functional  use.  It  is 
easy  to  conceive  that  the  functional  action  takes  place  more  readily  in 
some  lines  than  in  others,  and  these  lines  of  easiest  action  are  often 
spoken  of  as  "  lines  of  least  resistance,"  and  are  said  to  determine  the 
form  of  the  reflex  action.  In  its  simplest  form  a  "  reflex  arc  "  con- 
sists of  two  neurons,  a  centripetal  and  a  centrifugal,  between  the 
dendrites  of  which  the  transformation  of  the  impulse  takes  place. 
More  powerful  stimulation  brings  also  into  action  the  dendrites  of  the 
centripetal  neuron  whicb  are  associated  with  other  neurons  than  the 
centrifugal  already  mentioned,  and  hence  conduces  to  a  wider  area  of 
motor  discharge. 

The  stronger  (within  limits)  the  afferent  stimulus,  the  more  widely 
does  the  process  spread  in  the  centre,  and  the  more  extensive  is  the 
movement  produced.  Thus  a  slight  touch  on  the  sole  may  cause  only 
a  movement  of  the  foot,  while  a  prick  may  canse  a  movement  of  the 
whole  limb.  At  the  same  time  there  is  much  more  than  degree  involved 
in  the  difference  between  these  two  stimuli.  The  impression  that  is  felt 
as  pain  causes  a  quick  simple  movement  of  the  part,  in  which  chiefly 
the  flexors  are  involved.  It  is  a  movement  obviously  to  withdraw  from 
barm,  and  it  is  proportioned,  in  energy  and  extent,  to  the  intensity 
of  the  stimulation.  To  this,  however,  there  is  one  limitation : — a 
strong  impression  of  pain  may  prevent  all  reflex  movement  by 
exerting  an  inhibitory  influence  on  the  centres.  On  the  other  hand, 
a  tactile  impression  causes  a  much  more  complex  movement,  which 
does  not  bear  a  simple  relation,  in  extent  and  energy,  to  the  degree 
of  the  sensory  impulse,  but  is  also  influenced  by  the  quality  of  the 
impression  at  the  place  at  which  it  is  produced.  Thus  the  reflex  move- 
ment produced  from  the  palm  or  sole  is  especially  elaborate ;  it  may 
present  an  indication  of  the  central  relations  by  which  the  purposed  use 
of  the  extremities  is  facilitated.  The  effect  of  all  painful  impressions 
is  similar,  whether  these  are  produced  through  the  nerves  of  the  skm 
or  those  of  deeper  structures.  The  reflex  action  caused  by  pinching 
a  tendon  is  just  the  same  as  that  caused  by  a  pinch  of  the  skin  over 


240  SPINAL    COED. 

the  tendon.  Even  impressions  from  the  afferent  muscle-nerves  cause 
a  similar  reflex  movement.  Thus  there  is  no  real  distinction  between 
the  deep  and  superficial  true  reflex  action. 

The  muscle-reflex  action  which  is  assumed  to  exist  between  the 
afferent  and  efferent  nerves  of  a  muscle  (and  to  underlie  the  myo- 
tatic  irritability  which  permits  the  knee-jerk,  &c.)  has  been  already 
considered  in  discussing  the  theory  of  this  irritability.  It  is  appa- 
rently a  uniformly  simple  relation  between  the  two  sets  of  nerves 
for  the  same  part  of  a  muscle ;  the  reflex  action  is  confined  to  these, 
and  does  not  spread  through  a  wider  extent  of  the  grey  matter,  as 
that  from  the  skin  frequently  does.  When  a  muscle  is  made  tense, 
or  its  tendon  is  tapped,  the  mechanical  stimulation  influences  all  the 
afferent  nerves  or  all  the  muscular  fibres,  and  the  whole  muscle  neces- 
sarily responds.  The  motor  ganglion-cells  of  the  anterior  horn  must 
form  part  of  the  centre  for  each  form  of  reflex  action,  but  that 
portion  of  the  centre  which  intervenes  between  the  afferent  nerves 
in  the  posterior  root  and  the  motor  cells  must  be  distinct  in  part 
or  altogether.  Beyond  this  we  can  say  nothing  of  the  relation  of 
the  two  centres. 

These  reflex  centres  are  subject  to  control  by  higher  centres,  as  are  all 
lower  centres  in  the  nervous  system.  All  true  reflex  action,  e.  g.  that 
from  the  skin  excited  by  a  touch  or  prick,  is  controlled  and  kept  mode- 
rate by  an  influence  from  the  brain,  for  it  becomes  excessive  when  the 
influence  of  the  brain  is  stopped,  as  by  a  lesion  interrupting  the  spinal 
cord;  it  is  increased  in  all  parts  that  are  connected  with  the  cord 
below  the  lesion.  We  have  no  precise  knowledge  of  the  seat  of  the 
controlling  centre  or  of  the  path  by  which  the  control  is  exerted. 
In  frogs,  however,  the  restraint  seems  to  be  exerted  by  the  optic 
lobes ;  in  man  it  is  less  likely  to  be  exerted  by  the  homologous  corpora 
quadrigemina  than  by  the  optic  thalamus.  The  mechanism  must, 
however,  be  complex,  since  the  restraining  influence  may  be  increased 
by  disease  in  some  parts  of  the  brain ;  many  cerebral  lesions  which 
cause  hemiplegia  cause  also  a  diminution  of  cutaneous  reflex  action 
on  the  paralysed  side.  The  effect  seems  to  indicate  that  the  cerebral 
centre  which  controls  reflex  action  is  itself  habitually  controlled  by  a 
higher  centre,  perhaps  in  the  cortex.  When  this  is  destroyed,  or  the 
path  from  it  is  interrupted,  the  controlling  centre  passes  into  a 
condition  of  increased  activity,  and  the  reflex  action  is  lessened. 
This  is  a  theory  only,  but  it  seems  impossible  otherwise  to  explain 
the  facts. 

The  muscle-reflex  centres  seem  also  to  be  under  the  control  of  other 
centres,  because,  if  there  is  disease  of  the  cord  higher  up,  they  also  pass 
into  a  condition  of  increased  activity.  But  the  phenomena  of  their 
disturbance  present  three  im|iortant  points  of  difference  from  those  of 
common  (e.  g.  cutaneous)  reflex  action.  (1)  We  know  with  certainty 
the  path  disease  of  which  determines  their  excess :  it  is  the  pyramidal 
tract.     (2)  Disease  of  the  brain  does  not  cause  a  permanent  diminu- 


FUNCTIONS.  241 

tion  in  the  action  of  the  muscle-reflex  centres,  as  it  so  frequently  does 
in  the  case  of  the  centres  for  cutaneous  reflex  action.*  On  the 
contrary,  disease  of  the  pyramidal  fibres  in  the  brain  causes  the  same 
excess  of  this  form  of  reflex  action  as  does  disease  of  the  pyramidal 
fibres  in  the  cord.  (3)  The  excess  does  not,  as  a  rule,  quickly  follow 
an  interruption  of  the  path  from  the  brain,  as  does  the  excess  of 
superficial  reflex  action.  There  may,  indeed,  be  an  immediate,  usually 
temporary,  excess,  coinciding  with  "  early  rigidity  "  from  the  irritation 
of  the  pyramidal  fibres,  but  the  permanent  excess  comes  on  at  the  end 
of  a  week  or  ten  days,  and  gradually  increases. 

The  explanations  maybe  these.  The  slowness  with  which  the  reflex 
action  usually  becomes  excessive  is  conspicuous,  and  its  significance 
must  be  considerable.  The  lesion  of  the  pyramidal  fibres  causes  their 
degeneration  below  the  lesion,  and  this  must  involve  their  termination 
in  the  grey  matter.  The  excess  of  action  of  the  centres  may  be  the 
result  of  the  degeneration  of  the  terminal  structures,  which  may  con- 
stitute a  controlling  mechanism  that  normally  restrains  the  activity  of 
the  muscle-reflex  centres.  The  interval  which  elapses  before  the 
excess  is  manifested  may  be  due  to  the  fact  that  there  is  little 
tendency  to  increased  activity ;  that  but  slight  control  is  habitually 
exercised  or  needed ;  and  that  the  capacity  for  over-action  is  gradually 
developed  and  gradually  increases — increasing  capacity  being  deve- 
loped by  the  increased  activity. 

It  is  probable  that  the  voluntary  impulses  pass  from  the  termina- 
tion of  the  pyramidal  fibres  to  the  motor  cells  through  structures  of 
which  this  muscle-reflex  centre  forms  part,  and  that  therefore  its 
elements  must  be  in  part  excited  to  action  by  pyramidal  impulses — a 
conclusion  quite  compatible  with  a  restraining  influence  exerted  by 
these  fibres  or  their  endings  when  no  volitional  impulses  descend 
them. 

The  chief  local  forms  of  superficial  and  muscle-reflex  action  have 
been  ab-eady  described  (see  p.  19,  et  seq.).  Their  centres  are  found  in 
the  corresponding  segments  of  the  cord  shovm  in  the  table  at  p.  252. 

Adaptation  to  Postuee. — Every  difference  in  posture  involves  a 
difference  in  the  distance  between  the  attachments  of  the  muscles  of 
the  part.  When  the  muscles  are  at  rest  they  present  always  a  certain 
state  of  "  tone,"  which,  being  equal  in  every  posture,  must  vary  auto- 
matically with  every  change  of  posture,  however  brought  about.  In 
passive  movements  the  muscles  that  are  elongated  and  those  that  are 
shortened  adapt  themselves  to  the  change  as  it  proceeds.  Apparently 
the  increased  tension  on  the  former  makes  them  yield  to  the  move- 

•  A  very  rare  exception  to  this  rule  will  be  mentioned  when  the  functions  of  the 
brain  are  considered.  Moreover  the  knee-jerk  is  abolished  in  some  cases  of  tumour 
of  the  cerebellum,  a  phenomenon  to  be  remembered  in  connection  with  the  fact 
that,  while  one  part  of  the  afferent  impulses  from  the  muscles  acts  on  the  cord, 
another  part  apparently  passes  up  to  the  cerebellum.  See  the  account  of  co> 
ordination  of  movement  on  the  next  page. 

VOL.  I.  16 


242  SPINAL    COED. 

ment,  and  tlie  lessened  tension  on  the  latter  makes  them  contract,  each 
set  thus  preserving  its  proper  tone.  The  tone  of  a  muscle  seems  to 
be  produced  by  a  reflex  process,  due  to  a  certain  state  of  the  motor 
cells  reg-ulated  by  the  impressions  from  the  afferent  muscle-nerves. 
These,  as  we  have  seen,  are  produced  by  two  mechanical  influences, 
the  lateral  pressure  of  the  fibres  (as  in  the  pain  of  cramp)  and  the 
tension  on  the  muscle.  An  increase  of  the  latter,  in  passive  move- 
ment, seems  to  lessen  the  activity  of  the  cells  and  permit  the  fibres  to 
elongate ;  a  diminution  increases  the  activity  of  the  cells  and  causes 
the  fibres  to  shorten.  It  further  appears  that  the  tone  of  a  muscle  is 
influenced  through  the  spinal  cord  by  the  condition  of  its  antagonists. 
Thus  the  knee-jerk  may  be  increased  by  cutting  the  sciatic  nerre — i.  e. 
the  nerve  to  the  flexors,  or  diminished  by  stimulating  it.  An  increase 
similarly  results  when  the  hamstring  muscles  (the  antagonists  of  the 
quadriceps)  are  relaxed  by  flexion  of  the  knee.* 

Co-OEDiNATioN  OF  MOVEMENT. — The  co-ordination  of  muscular 
contractions,  which  results  in  the  ordination  or  ordering  or  arrange- 
ment of  movement,  has  been  already  described  (p.  7),  so  far  as  its 
general  features  are  concerned.  We  cannot  ascribe  to  the  spinal  cord 
any  process  that  can  be  designated  the  arrangement  of  movement ; 
since  the  muscular  contractions  correspond  to  the  activity  of  the  motor 
cells  of  the  cerebral  cortex,  and  to  the  corresponding  impulses  along 
the  pyramidal  fibres.  These  determine  the  initial  activity  of  the 
motoi-  cells  of  the  cord,  both  for  the  muscles  that  move  a  part  and  for 
those  that  oppose  the  action  of  the  others  and  give  steadiness  and  uni- 
formity to  the  movement.  But  these  impulses  from  the  brain  descend 
on  spinal  cells  that  are  not  in  a  passive,  inactive,  functionless  state. 
Such  a  state  probably  never  exists,  for  even  when  there  is  no  voluntary 
activity  there  must  be  the  activity  on  Avhich  the  tone  depends, — that, 
as  we  have  just  seen,  must  exist  in  all  postures  of  the  limbs.  This 
state  of  the  cells  preceding  voluntary  activity  is  determined  by  the 
afferent  impulses,  chiefly  by  those  from  the  muscles.  Through  these 
the  muscles  are  adapted  to  postiire,  and  in  the  mechanism  for  adapta- 
tion to  postuxe  we  may  trace  also  a  mechanism  for  part  at  least  of  the 
co-ordination  effected  in  the  spinal  cord.  This  seems  to  consist  of 
two  parts.  (1)  An  automatic  variation  in  the  state  of  muscles,  per- 
mitting the  ordered  performance  of  intended  movements.  The  state 
of  the  afferent  impulses  acting  from  the  muscle  on  the  motor  cells 
must  be  at  once  altered  by  the  effect  of  the  voluntary  activity  of  the 
muscles,  since  this  will  alter  the  impulses  in  the  muscle-nerves  by  the 
changed  and  changing  pressure  and  tension  on  the  tissues  in  which 
these  nerves  end — the  pressure  of  the  contracting  and  widening  fibres 
and  the  tension  in  the  antagonists  that  oppose  the  motion,  but  do  not 
prevent  it,  and  are  made  more  tense  by  the  muscles  they  are  opposing. 
These  impulses  pass  to  the  cord,  and  there  act  on  the  motor  cells,  and 
influence  their  state  in  a  way  that  we  can  at  present  only  guess  at,  but 
•  See  Shemng-'on,  'Brit.  Med.  Journ.,'  1893,  vol.  ii,  p.  685. 


FUNCTIONS.  243 

which,  must  be  of  the  utmost  importance.  There  must  of  necessity 
be  a  definite  relation  between  certain  states  of  the  muscle  and  the 
strength  of  afferent  impulse  produced  thereby.  If  we  conceive  that 
an  increase  in  the  impulse  (making  it  greater  than  corresponds  to  the 
degree  of  activity  of  the  motor  cells),  such  as  must  resiilt  from  the 
increased  tension  on  the  muscle,  lowers  the  activity  of  those  cells,  we 
can  understand  the  way  in  which  the  contracting  antagonists  relax  to 
permit  movement  which  tends  to  occur.  As  soon  as  the  resistance 
begins  to  yield,  the  tension-stimulus  will  be  increased,  and  the  total 
afferent,  impulse  become  disproportionately  great  in  relation  to  the 
degree  of  activity  of  the  cells  ;  as  a  result,  this  activity  is  lessened  and 
the  muscles  yield.  This  involves  a  constant  regulation  of  the  activity 
of  the  various  motor  cells  by  the  afferent  impulses.  A  like  but 
opposite  effect  must  be  produced  on  the  cells  of  the  muscles  producing 
the  movement.  This  effect  on  the  cells  will,  of  course,  blend  with  that 
of  the  voluntary  impulse  from  the  brain  that  is  causing  the  movement. 
Thus  the  spinal  muscle-reflex  process  permits  the  desired  movement 
to  take  place,  as  it  could  not  do  if  the  state  of  the  cells  were  not  thus 
automatically  regulated.  (2)  Relations  between  the  various  reflex 
processes  are  doubtless  established  by  the  repetition  of  movements, 
and  thus  planned  mechanisms,  as  it  were,  are  established  in  the  cord  ; 
within  their  range  the  various  movements  occur.  The  state  of  the 
muscles  in  standing,  for  instance,  is  almost  perfectly  reproduced  by 
the  extensor  spasm  that  occurs  when  the  muscle- reflex  processes  are  in 
great  excess.  Thus  we  have  a  mechanism  by  which  a  relatively  simple 
voluntary  impulse  may  produce  an  extensive  co-ordinated  movement. 
But  what  we  can  discern  shows  that  we  must  regard  spinal  co-ordina- 
tion as  essentially  a  reflex  process,  chiefly  due  to  the  impulses  from  the 
muscles.  The  correctness  of  this  view  can  hardly  be  doubted  when 
we  come  to  see  its  perfect  harmony  with  the  phenomena  and  the  con- 
ditions of  spinal  inco-ordiaation. 

Stm  it  must  be  always  remembered  that  this  spinal  process  is  of 
the  nature  of  a  subordinate  mechanism,  determining  the  precision  of 
form  rather  than  the  actual  arrangement  and  order  of  the  musctdar 
contraction.  This  is  done  by  a  higher  process,  a  co-ordination  which 
consists  in  the  form  of  action  of  the  motor  cells  of  the  cerebral 
cortex  that  is  produced  by  stUl  higher  volitional  processes.  These, 
however,  as  we  shall  see,  are  themselves  subject  to  a  like  influence  and 
the  seat  of  an  analogous  regulation,  chiefly,  it  is  probable,  by  impulses 
proceeding  from  the  cerebellum,  which  are  also  due  to  the  varying 
excitation  of  the  muscle-nerves.  That  the  nerve-fibres  which  pass  up 
the  posterior  median  columns  conduct  impulses  from  the  muscles  that 
determine  cerebral  co-ordination  scarcely  admits  of  doubt.  Interrup- 
tion of  this  path  in  any  part  of  its  course  causes  a  defect  of  co- 
ordination, which  resembles  that  due  to  cerebellar  disease  with  sufficient 
closeness  to  add  weight  to  other  facts  suggesting  that  it  is  through 
the  cerebellum  that  the  impulses  carried  by  this  path  act  on  the 


244.  SPINAL    CORD. 

cortex,  regulating  and  determining  the  activity  of  its  cells  and  tlie 
resulting  movement.*  The  impulses  may  reach  the  cerebellum,  as 
we  have  seen,  from  the  grey  matter  of  the  post-pyramidal  nucleus  in 
which  these  fibres  end.f  But  this  primary  co-ordination  is  not  a 
function  of  the  cord,  although  it  may  be  lost  in  consequence  of 
disease  of  the  cord.  The  direct  cerebellar  tract  may  constitute  a 
similar  path,  but  of  this  we  have  only  the  evidence  of  destination  and 
analogy. 

It  is  probable  that  the  mechanisms  just  alluded  to,  as  constituted 
by  the  functional  relations  between  the  motor  and  sensory  structures 
in  the  cord,  very  much  facilitate  the  execution  of  many  movements, 
and  especially  of  those  that  are  the  more  simple  in  their  character. 
The  less  variation  a  movement  is  capable  of,  or  needs,  the  more  largely 
may  the  spinal  mechanism  be  concerned  in  its  arrangement ;  and  hence 
such  actions  as  standing  and  walking  may  be  effected  through  a 
comparatively  small  number  of  the  pyramidal  fibres,  and,  as  we  shall 
see,  they  may  still  be  performed  when  there  is  disease  that  would 
prevent  a  more  elaborate  action.  It  is  also  probable  that  in  animals 
such  spinal  mechanisms  are  developed  to  a  greater  extent  than  in 
man,  and  care  must  be  taken  in  drawing  inferences  from  one  to  the 
other. 

The  impulses  from  the  muscles  that  determine  the  muscle-reflex 
action  and  the  spinal  co-ordination,  must  pass  by  nerves  that  end  in 
the  grey  matter  and  come  into  relation  with  nerve- cells,  at  or  near  the 
level  at  which  they  enter  the  cord.  We  do  not  know  whether  it  is 
through  these  fibres,  or  through  those  that  pass  upwards  without 
interruption,  that  the  impulses  are  conveyed  which,  when  excessive  in 
degree,  give  rise  to  muscular  sensations.  But  attention  may  again  be 
called  to  the  important  evidence,  afforded  by  such  sensations,  of  the 
abundance  of  upward  impulses  of  which  we  are  normally  unconscious. 
These  nerves,  it  will  be  remembered,  begin  in  the  connective  tissue 
of  the  muscles,  and  probably  also  in  the  muscle-spindles,  and  the  acute 
pain  of  cramp,  and  the  muscular  tenderness  in  such  affections  as 
multiple  neuritis,  afford  indirect  evidence  of  impulses  which  we  do  not 
usually  observe,  and  might  almost  doubt ;  while  the  manner  in  which 
such  pain  is  excited  is  very  clear  evidence  of  the  sensitiveness  of  the 
nerves  to  the  modes  of  stimulation  that  have  been  assumed  as  effective. 
We  have  already  seen  (p.  17)  that  it  is  through  such  unfelt  impulses 
that  we  probably  derive  the  chief  'part  of  our  conceptions  of  posture,  a 
subject  that  must  again  be  considered  in  connection  with  the  brain. 

Inflitence  on  Nijteition. — The  nutrition  of  the  muscles  is  under 

*  This  has  been  experimentally  confirmed  by  Bechterew,  who  finds  that  section 
of  the  posterior  columns,  in  various  animals,  above  the  lumbar  enlargement  causes 
a  defect  of  co-ordination  similar  to  that  produced  by  loss  of  the  cerebellum  (Beclite- 
rew,  •  Wjestnik  psich.  i.  nevropafc.,'  1889,  vii,  1 ;  and  '  Neur.  Ceiitralbl.,*  1890,  p.  82.) 

t  A  connection  since  confirmed  by  Brosset,  *  Des  Connexions  du  Cervelet,*  Paris.. 
1891. 


FUNCTIONS.  245 

the  coutrol  of  the  anterior  grey  matter,  and  probably  of  the  motor 
nerve- cells.  The  influence  is  exerted  through  the  motor  nerves,  and 
indeed  consists  in  the  normal  integrity  of  the- fibres  (see  p.  29). 

The  nutrition  of  the  bones  smd  joints  is  also  under  the  control  of  the 
cord,  and  probably  is  exerted  through  the  posterior  roots.  It  is  true 
the  grovrth  of  the  bones  is  hindered  when  the  anterior  cornua  are 
diseased,  but  no  atrophic  change  seems  then  to  occur  in  the  osseous 
structure,  and  the  influence  of  the  paralysis  may  be  indirect.  A  true 
trophic  change,  and  an  alteration  in  the  joints,  may  occur  in  locomotor 
ataxy,  in  which  the  disease  is  of  the  posterior  nerve-fibres. 

The  nutrition  of  the  skin  seems  also  to  depend  on  nerves  that  have 
their  coxirse  in  the  posterior  roots.  The  clearest  fact  is  that  irritation 
■of  the  nerve- structures  has  far  more  effect  than  simple  loss  of  function. 
It  is  when  the  cord  or  nerve-roots  are  the  seat  of  irritative  inflam- 
mation that  the  most  acute  trophic  changes  occur,  while  mere  degene- 
ration of  these  nerves  induces  slower  and  slighter  changes  (see  p.  28) . 

In  a  dog  in  which  the  spinal  cord  below  the  cervical  region  was 
removed  piecemeal,  trophic  changes  in  the  skin  followed  the  section, 
but  not  the  subsequent  removal.  It  is  hence  doubtful  whether 
trophic  fibres  actually  take  origin  (i,  e.  have  "  centres  ")  in  the  cord ; 
in  all  cases  it  is  difficult  to  exclude  the  effects  of  the  fall  of  blood- 
pressure.* 

"Visceral  Contkol. — Although  the  viscera  are  under  the  immediate 
<jontrol  of  the  sympathetic  system  of  nerves,  they  are  related  to  centres 
in  the  spinal  cord,  and  it  is  from  these  centres  that  the  controlling 
influence  is  really  derived,  probably  by  means  of  the  finer  fibres  of 
the  anterior  roots.  The  relation  is  the  most  direct  and  important, 
so  far  as  concerns  the  diseases  of  the  spinal  cord,  in  the  case  of 
the  organs  over  which  the  will  has  an  influence,  the  rectum  and  the 
hiadder.  The  centres  for  these  are  in  the  lumbar  enlargement,  but  we 
do  not  know  their  exact  position.  They  are  probably  complex  reflex 
centres.  We  can  best  understand  their  action  by  studying  them  when 
voluntary  influence  is  lost. 

The  centre  for  the  sphincter  ani  is  the  more  simple,  but  the  system 
of  action  of  each  is  probably  similar.  In  the  wall  of  each  viscus  we  have 
muscular  fibres  to  expel  the  contents,  and  at  the  mouth  a  sphincter 
arrangement  to  prevent  continuous  evacuation.  Fseces  or  air  in  the 
rectum,  and  urine  in  the  bladder,  may  excite  the  lumbar  centre, 
and  cause  two  effects — contraction  in  the  wall  and  relaxation  of  the 
sphincter.  This  process  can  be  controlled  by  the  will  to  a  consider- 
able extent,  although  we  are  still  ignorant  of  the  precise  mode  in  which 
the  voluntary  influence  is  exei'ted.  But  if  the  volitional  path  in  the 
<iord  is  interrupted  above  the  lumbar  centres,  the  will  can  no  longer 
control  the  reflex  processes ;  as  soon  as  faeces  irritate  the  rectum  they 
are  expelled  by  the  reflex  mechanism ;  as  soon  as  a  sufficient  quantity 
of  \irine  accumulates  in  the  bladder  a  reflex  contraction  of  the  detrusor 

*  Goltz  and  Ewald,  'Arch,  f,  d.  gesammt.  Physiol./  1896. 


246 


SPJNAL    CORD. 


and  relaxation  of  tlie  spMncter  cause  its  escape.  Tlie  affection  of  the 
voluntary  path  for  the  sphincter  is  not  always  proportioned  to  that 
for  the  legs.  If  the  damage  to  the  cord  inrolves  also  the  sensory  tract, 
the  patient  is  unconscious  of  the  action  of  the  bladder  or  bowel.  If 
the  sensory  tract  is  unaffected,  the  patient  is  aware  of  the  process,  but 
cannot  control  it.  It  is  often  said  that  there  is  permanent  relaxation 
of  the  sphincters,  but  this  is  true  only  when  the  lumbar  centres  are 
inactive  or  destroyed.  In  this  condition  evacuation  occurs  as  soon 
as  faeces  or  urine  enter  the  bowel  or  rectum.  The  urine  escaj^es 
continuously,  instead  of  being  expelled  at  intervals.  The  condition  is 
less  obvious  in  the  case  of  the  rectum,  because  there  is  no  such  con- 
tinuous passage  of  faeces  into  the  rectum  as  there  is  of  urine  into  the 
bladder.  We  may,  however,  distinguish  between  the  two  states  of  the 
rectum  by  the  introduction  of  the  finger.  If  the  lumbar  centre  is 
inactive,  there  is  a  momentary  contraction,  due  to  local  stimulation  of 
the  sphincter,  and  then  permanent  relaxation.  If,  however,  the  reflex 
centre  and  motor  nerves  from  it  are  intact,  the  introduction  of  the 
fingei  is  followed,  first  by  relaxation,  and  then  by  gentle,  firm,  tonio 
contraction.      I  have  verified   this   by  introducing  an  india-rubber 


3IQ 


I 


II 


II 


liiii! 


Ill 


IB!        E 


Fig-.  85. — Teacings  of  the  Action  op  the  Sphincteb  Ani. 

The  vertical  lines  represent  seconds  of  time. 
Effect  on  contraction  of  sphincter  of  the  injection  into  the  rectum  of  a  small 
quantity  of  air  at  *.  a,  fall  in  pressure  due  to  the  inhibition  of  the  contrac- 
tion;  J,  rise  due  to  the  slowly  returning  contraction.  2.  Effect  of  the  intro- 
duction of  the  instrument,  a,  sudden  rise  of  lever  at  moment  of  introduction, 
due  to  the  exposure  of  the  instrument  to  the  pressure  of  the  sphincter  (the  top 
of  this  line  represents  the  deo^ree  of  previous  contraction) ;  5,  initial  rise  due  to 
increased  contraction;  c,  fall  from  partial  inhibition;  d,  subsequent  contrac- 
tion, rising  to  a  greater  degree  than  the  initial  contraction,  iimi  subsequently 
falling  slifjlitly.  3.  Effect  of  cough,  a,  pressure  of  tonic  contraction  of 
spliiucter  (the  slight  irregularities  are  due  to  pulse-waves);  h,  fall  in  pressure, 
due  to  the  movement  of  the  instrument  by  the  cough;  c,  initial  contraction ; 
<Z,  relaxation  of  inhibited  sphincter ;  e,  e,  e,  rhythmical  variations  in  subsequent 
rise.     (From  the  '  Proc.  Royal  Society,'  1877.) 


cylinder  instead  of  the  finger,  and  registering  the  pressure  on  the 
cylinder  by  connecting  it  with  a  recording  apparatus.  The  relaxation 
is  then  found  to  be  preceded  by  a  very  slight  brief  contraction,  and 


FUNCTIONS. 


247 


to  be  followed  by  unbroken  tonic  contraction.  Tlie  relaxation  may 
also  be  readily  produced  by  any  impression  on  the  raucous  membrane 
above  tbe  sphincter.     Fig.  85  shows  some  of  the  tracings  obtained. 

The  action  of  the  bladder  mechanism  can  be  best  understood  by 
assuming  that  the  motor  centre  really  consists  of  two  parts,  one  (ms, 
Fig.  86)  maintaining  the  contraction  of  the  sphincter,  the  other  (md) 
exciting  the  contraction  of  the  detrusor  fibres,  and  that  these  two 
parts  are  antagonistic ;  when  one  acts  the  other  is  inhibited.  Thus, 
in  normal  rest,  the  sphincter  centre  is 
active,  the  detrusor  at  rest. 

Apparently,  the  process  of  micturition 
is  effected  by  the  automatic  contraction 
of  the  wall,  when  the  sphincter  is  re- 
laxed. The  sphincter  is  certainly  under 
the  influence  of  the  will,  which  is  able 
to  maintain  and  increase  its  contraction, 
but  it  is  doubtful  whether  the  mere  ces- 
sation of  the  voluntary  influence  is  alone 
the  cause  of  its  relaxation.  The  elements 
of  the  process  can  be  best  discerned  when 
it  is  weakened  by  slight  disease.  It  is 
then  clear  that,  in  addition  to  the  cessa- 
tion of  voluntary  contraction,  the  relaxa- 
tion is  due  to  the  sensory  impulses  from 
the  mucous  membrane  at  the  neck  of  the 
bladder,  but  these  do  not  seem  to  in- 
hibit the  spinal  centre  directly.  This 
only  becomes  inactive  when  the  afferent 
impulses  act  on  the  sensory  centres  in  the 
brain,  and  it  may  be  essential  for  the 
influence  on  these  centres  to  be  increased 
by  the  lowering  of  resistance  involved  in 
conscious  "attention."  K  the  sensation 
is  not  attended  to  no  relaxation  may 
occur.  The  effect  may  be,  indeed,  due 
to  an  automatic  cerebral  influence,  main- 
taining the  action  of  the  spinal  centre, 
which  is  only  "taken  off"  when  the 
sensory  impression  causes  its  full  effect 
on  the  cerebral  processes.  The  elements 
of  the  mechanism  are  scarcely  to  be  discerned  in  health,  but  may 
under  exceptional  conditions  be  clearly  perceived. 

Although  no  other  part  of  the  alimentary  canal  is  under  voluntary 
influence,  all  parts  are  probably  related  to  the  spinal  cord,  by  means 
of  the  connection  between  the  sympathetic  system  and  the  nerve- 
roots.  Constipation  is  extremely  common  in  disease  of  the  spinal 
cord,  and  is  often  greater  than  can  be  accounted  for  by  the  mere 


Fig.  86.— Diageam  showing 
Probable  Plan  of  the 
Centre  ron  Mictfeition. 

MT,  motor  tract  J  ST,  sensory 
tract  ill  the  spinal  cord;  MS, 
centre,  and  ms,  motor  nerve 
for  sphincter;  md,  centre, 
and  md,  motor  nerve  tor  de- 
trusor; s,  aH'ereiit  nerve  from 
mucous  membrane  to  s,  sen- 
sory portion  of  centre;  B, 
bladder.  At  r  the  condition 
during  rest  is  indicated,  the 
sphincter  centre  in  action, 
tlie  detrusor  centre  not  act- 
insr.  At  a  the  condition 
during  action  is  indicated, 
the  sphincter  centre  inhibited, 
the  detrusor  centre  acting. 


248  SPINAL    CORD. 

loss  of  power  of  the  abdominal  muscles.  The  pain  in  tlie  back  that 
is  so  common  in  disease  of  the  stomach  has  been  regarded  as  a 
reflected  pain,  due  to  this  connection. 

The  uterine  functions  are  probably  independent  of  the  cord  in 
greater  degree  than  those  of  any  other  organs  under  the  influence  of 
the  sympathetic.  The  function  of  menstruation  goes  on  independently 
of  cord  disease,  and  may  be  regular  although  there  is  absolute  arrest 
of  aU  conduction,  sensory  and  motor.  Pregnancy  and  labour  may 
proceed  in  normal  course,  in  spite  of  such  disease  of  the  cord  as  causes 
complete  motor  paraplegia. 

Sexual  Functions. — The  activity  of  the  sexual  organs  depends  on 
the  integrity  of  the  reflex  loop  to  and  from  a  special  centre,  also 
situated  in  the  lumbar  enlargement,  but  the  due  action  of  this  centre 
depends  on  cerebral  (psychical)  as  well  as  on  reflex  influences.  Dis- 
ease of  the  centre,  or  of  the  nerves  leading  to  or  from  it,  abolishes 
sexual  action.  The  sexual  reflex  is,  however,  one  of  the  cutaneous 
reflexes,  and  it  shares  the  condition  of  these  rather  than  of  the  muscle- 
reflex  processes.  It  has  been  stated  that  in  tabes  loss  of  sexual  power 
is  associated  with  loss  of  tactile  sensibihty  of  the  glans  penis.* 

The  centre  is  probably  double,  and  its  action  is  impaired  by  inter- 
ference with  either  half.  When,  by  disease  higher  up,  the  connection 
with  the  psychical  centres  is  interrupted,  the  sexual  act  cannot  be 
perfectly  performed.  If  the  path  from  the  controlhng  centre  (p.  20) 
is  unimpaired,  the  reflex  sexual  processes  are  not  in  excess,  and  may 
even  be  diminished  ;  but  if  the  path  from  this  controlling  centre  is  also 
interrupted,  the  reflex  sexual  processes  are  in  excess  like  the  other 
superficial  reflexes,  and  priapism  results.  .  If  the  reflex  centre,  or 
connecting  paths,  are  partially  diseased,  the  sexual  process  may  be 
impaired  and  imperfect,  but  not  abolished. 

Vaso-motor  Centres. — The  sympathetic  nerves  to  the  vessels  are 
influenced  from  the  spinal  cord.  It  is  probable  that  the  path  is  by 
the  fine  fibres  of  the  anterior  roots,  and  that  most  of  the  constrictor 
fibres  leave  the  cord  between  the  third  dorsal  and  the  second  lumbar, 
while  the  dilator  fibres  are  more  widely  scattered,  many  arising  in  the 
upper  dorsal  region,  while  others  leave  the  cord  in  its  lumbar  and 
sacral  portions  ("pelvic  outflow").  Some  facts  of  disease  suggest 
that  the  subsidiary  vaso-motor  centres  are  situated  in  the  intermediate 
grey  matter;  and  this  conclusion  is  supported  by  the  important 
researches  of  Gaskell,  which  refer  the  function  to  the  small  cells  of  the 
intermedio-lateral  tract,  which  he  traces  upwards  to  the  vaso-motor 
centre  in  the  medulla.  The  action  of  the  vaso-motor  nerves  may  be 
deranged  by  disease  of  the  cord.  It  is  probable  that  such  a  relation 
exists  between  all  parts  of  the  vaso-motor  system  of  nerves  and  the 
cord,  and  that  the  relation  obtains  between  each  segment  of  the  cord 
and  the  vascular  nerves  to  the  corresponding  part  of  the  trunk  and 
limbs.  This  derangement  in  disease  may  take  a  part  in  the  production 
*  See  Brown,  '  Lancet,'  1898. 


FUNCTIONS.  249 

of  tie  changes  in  nutrition,  but  it  is  probably  not  tbe  sole  mechanism 
by  which  these  changes  are  produced. 

There  is  a  special  connection  between  the  sympathetic  and  the 
cervical  region  of  the  spinal  cord.  This  region  contains  the  path  to 
the  visceral  neiwes  that  control  'the  sugar-forming  functions  of  the 
liver.  The  lower  part  of  the  cervical  enlargement  contains  also  an 
important  centre  which  controls  the  sympathetic  nerves  for  the  vessels 
of  the  head  and  face.  The  path  of  central  influence  for  the  radiating 
fibres  of  the  iris  passes  also  from  the  region  of  the  third  nerve  nucleus, 
down  the  cervical  cord,  by  the  lowest  cervical  or  first  dorsal  nerve,  to 
the  sympathetic,  and  then  ascends  the  cervical  trunk,  and  passes  along 
the  fibres  which  accompany  the  internal  carotid  artery  to  the  nasal 
division  of  the  fifth  cranial  nerve.  In  this  the  fibres  run  for  some 
little  distance,  ultimately  reaching  the  dilator  muscle  as  the  long 
miliary  nerves.  Fibres  also  pass  from  the  cervical  cord  to  the  sym- 
pathetic, through  which  the  action  of  the  heart  is  accelerated. 

Mtjttjai,  Relation  of  the  Functions  of  the  Spinal  Coed. — It 
may  be  well  to  present,  in  the  form  of  a  table  (p.  252),  the  relation  of 
the  motor  and  reflex  functions  of  the  cord,  as  far  as  our  knowledge  of 
these  relations  extends.  Many  points  are  still  uncertain.  The  sensory 
relations  wiU  be  sufficiently  obvious  from  the  figures.  The  table  does 
not  need  any  detailed  explanation.  It  is  merely  a  comparative  collection 
of  the  facts  that  have  been  stated  in  the  preceding  pages.  We  shall 
find  the  value  of  this  collocation  of  functions  when  we  consider  the 
effects  of  transverse  lesions  of  the  cord  at  different  levels. 


250 


SPINAL    CORD. 


Fia.  87. 


Figs.  87  and  88. —  Dr.  Ilciiiy  Hcjid  lias  been  kind  enough  to  permit  the  use  of  these, 
researches,  mainly  on  eases  of  herpes  zoster  and  of  localised  spinal  cord  lesions.  It 
majority  are   approximately  so,  and,  in  any  case,  they  will  serve  as   a  substantial 


PFNOTIONS. 
Fia.  88. 


231 


riiuy  were  devised  by  him,  and  the  sensory  areas  are  marked  out  at  a  result  of  his 
Is  not  claimed  that  all  the  areas  are  absolutelj  correct.  It  if  believed  that  the 
basis  for  future  research. 


252 


SPINAL    COED. 


"Tdbie  showing  approximate  relation  to  the  spinal  nerves  of  the  motor  and  reflex 
functions  of  the  spinal  cord.  The  sensory  areas  are  indicated  with  sufficient 
clea/rnesR  in  Figs.  87  and  88. 


C.  1 


Sfcemo-mastoid, 

upper  neck 
muscles,  upper 
part  trapezius 


Lower  neck 

muscles,  middle -j 

part  trapezius 


Small  rotators  of  head. 
2  I    Depressors  of  hyoid  bone. 

Scaleni. 

Lev.  ang.  scapulae. 


BBFLEZ. 


^ 


Diaphragm 


4 
5 

m  J  Extensors  of  wrist  and  fingers 
8 


>    I  Pectoralis  (clavicular  part) 

\     j  Serratus 

>■    I  Plexors  of  elbow 

1  Pronators,  triceps 
J  Ext 


Deltoid  and 

other  shoulder 

•  muscles,  and  ■ 

sternal  part 

of  pectoralis 


>  Flexors  of  wrist  and  fingers 

"  1  Muscles  of  hand  • 

J)    1  J  Extensors  of  thumb  • 


Lower  part 
trapezius  and 
back  mascles 


2 
3 
4 
5 
6 
7 
8 
9 
10 
11 

L.l 


r 


Lumbar  muscles 

Peroneus,      C 

flexor  of  ankle,  J 

extensor  of    ] 

ankle         1 


} 


Intercostals        • 

Abdominal  miiscles 

Ileo-psoas      • 
Cremaster  , 


a  J    Flexors  of  hip    ,  , 

1  Extensors  of  knee  • 

^  J  Adductors  of  hip  . 

y  Extensors  and  abductors  of  hip 


.} 


Plexors  of  knee 


V  Intrinsic  muscles  of  foot  . 


2 
8 
4  y  Periuseal  and  anal  mascles. 

<i 


;i 


Dilatation  of 
pupil. 

Scapular. 


1 


Epigastri& 


>  Abdominal. 


Cremasteric. 


Gluteal. 
\Foot- 
J     clonus. 
Plantar. 


SYMPTOMS.  253 


SYMPTOMS  OF  BTSBASH  OF  TEF  SPINAL  COB,D :  INDICATIONS 
OF  TEE  SEAT   OF  TEE  LESION:  ANATOMICAL  DIAGNOSIS. 

The  symptoms  of  disease  of  the  spinal  cord  consist  in  derangement 
of  its  various  functions ;  the  loss  of  some,  the  exaltation  and  perver- 
sion of  others.  We  have  already  considered  these  functions,  and  the 
parts  of  the  cord  by  which  they  are  subserved.  We  may  now  briefly 
consider  the  general  character  of  their  derangement,  and  its  signifi- 
cance in  regard  to  the  position  of  the  disease.  The  combination  of 
symptoms  indicates  the  seat  of  the  lesion ;  we  infer  its  nature  from 
their  mode  of  development,  and  other  considerations,  which  will  be 
subsequently  discussed.  It  is  always  important  to  keep  these  two 
elements  of  the  diagnosis  distinct  in  the  mind. 

A  common  feature  of  the  symptoms  of  disease  of  the  spinal  cord  is 
their  bilateral  character.  This  depends  on  two  causes,  of  which, 
however,  only  the  first  is  peculiar  to  the  cord,  and  can  be  regarded  as 
the  special  cause  of  this  feature  of  its  diseases.  The  structures  in 
each  haK  of  the  spinal  cord  are  in  such  close  proximity,  that  any 
random  process,  such  as  haemorrhage  or  inflammation  or  pressure, 
readily  affects  both  halves — can,  indeed,  hardly  fail  to  do  so  in  some 
degree,  and  usually  to  such  an  extent  as  to  cause  the  bilateral  cha- 
racter of  the  symptoms  to  be  obtrusive.  Secondly,  morbid  processes 
that  commence  in  the  nerve-elements  and  affect  them  according  to 
their  function  ("  system  diseases  ")  usually  involve  the  corresponding 
structures  of  the  two  sides,  and  the  spinal  cord  contains  many  struc- 
tures that  are  liable  to  such  processes.  Hence  a  large  proportion  of 
the  diseases  of  the  spinal  cord  involve  both  sides  and  cause  symptoms 
that  have  a  corresponding  range.  The  leading  fact  that  results  from 
this  is,  that  the  characteristic  type  of  palsy  is  "  paraplegia."  The  word 
means  literally  paralysis  of  the  parts  beneath  or  beyond  a  certain 
locality,  and  involves  the  distinction  of  a  motionless  part  of  the  frame, 
which  no  doubt  arose  from  the  variable  extent  of  this,  in  comparison 
with  the  uniformity  of  the  region  affected  in  "hemiplegia."  But 
the  second  condition  causing  the  bilateral  character  of  the  symptoms 
is  shared  equally  with  those  outlying  parts  of  the  spinal  cord — the 
peripheral  nerves ;  and  as  this  condition  obviously  determines  a  stricter 
symmetry  than  does  the  first,  it  follows  that  this  character  is  not, 
alone,  of  much  significance,  and  is  indeed  least  significant  when  most 
complete.  In  the  affections  that  are  strictly  symmetrical,  therefore, 
other  features  must  also  be  taken  into  account. 

Another  character  of  spinal  symptoms,  not  shared  by  those  of  the 
nerves,  consists  in  their  variation  in  vertical  extent  according  to  the 
position  or  extent  of  the  lesion.  But  we  must  distinguish  two  classes 
of  symptoms  within  the  region  in  which  they  exist.  (1)  Those  which 
depend  on  the  interruption  of  the  conducting  path  to  or  from  the  brain. 


254  SPINAL    OORD. 

and  involve  the  whole  region  below  the  disease.  (2)  Those  which 
depend  on  damage  to  the  central  structures  in  the  cord  and  nerve- 
roots,  which  are  present  only  at  the  level  of  that  damage,  i.  e.  in  the 
parts  functionally  related  to  that  part  of  the  cord.  The  two  sets  of 
symptoms  may  be  distinguished  as  "  conducting  "  and  "  central ; "  but 
in  many  diseases  those  at  the  level  of  the  lesion  are  chiefly  due  to  the 
irritation  of  the  nerve-roots,  and  these  are  most  usefully  designated 
"root  symptoms." 

Disease  of  the  cord  does  not  always  cause  bilateral  symptoms. 
A.  little  consideration  will  show  that  this  must  be  so.  A  random 
process,  of  small  extent,  may  damage  one  side  only.  It  may  affect 
one  or  several  structures  on  one  side  or  all  of  them.  Nevertheless  it 
is  very  rare  for  such  a  process  to  spare  the  other  side  altogether,  and 
practically  no  lesion  affects  all  parts  of  one  side  without  damaging,  in 
some  degree,  the  other  side,  at  any  rate  for  a  time.  In  very  rare 
forms  of  traumatic  lesion,  however,  the  damage  to  the  other  side  is 
almost  imperceptible.  Again,  "  system  diseases,"  in  quite  exceptional 
cases  and  for  unknown  reasons,  are  limited  to  one  side ;  much  more 
frequently  they  may  affect  one  side  earlier  and  more  than  the  other. 
Such  unilateral  symptoms  indicate  a  condition  awkwardly  termed 
"  hemi-paraplegia." 

MoTOE  Symptoms. — Loss  of  motor  power  results  from  interference 
with  the  motor  path  in  any  part  of  its  intra-spinal  course, — ^pyramidal 
tracts,  anterior  grey  matter,  anterior  nerve-roots.  We  have  seen  that 
the  path  may  be  divided  into  two  segments,  upper  and  lower  (see 
p.  213).  The  spinal  cord  contains  a  considerable  portion  of  the  nerve- 
fibres  of  the  upper  segment,  and  their  lower  termination  in  the  grey 
matter,  but  only  the  commencement  of  the  lower  segment,  the  motor 
ganglion- cells,  and  root-fibres  proceeding  from  them.  The  chief  part 
of  the  lower  segment  is  outside  the  spine,  in  the  nerve-trunks.  So  far 
as  the  loss  of  power  is  concerned  the  effect  is  the  same,  in  whatever 
part  of  the  motor  path  the  interruption  is  situated;  but  the  other 
symptoms  that  accompany  the  loss  of  power  differ  very  much  accord- 
ing as  the  interruption  is  in  the  upper  or  lower  segment.  The  lower 
segment  influences  muscular  nutrition,  and  forms  part  of  the  path  of 
reflex  action.  Hence,  as  we  shall  presently  see,  the  muscles  waste,  and 
reflex  action  is  lost,  when  this  is  diseased;  but  when  the  upper 
segment  is  damaged  there  is  no  musctdar  wasting,  and  reflex  action, 
instead  of  being  lost,  is  commonly  increased.  The  symptoms  pvodiiced 
hyi  disease  of  either  segment  are  essentially  the  same,  whatever  part  of 
the  segment  is  diseased,  whether  the  cell  at  the  upper  extremity,  the 
fibre,  or  the  ramification  at  the  lower  extremity  of  the  fibre.  If,  for 
instance,  the  intra-muscular  nerve-endings  are  paralysed  by  curara  the 
symptoms  produced  are  the  same  as  if  the  motor  fibres  in  the  nerve- 
trunks  are  divided  or  their  cells  in  the  cord  are  destroyed.  Disease  of  the 
termination  of  the  upper  segment  in  the  grey  matter  of  the  cord  must, 
of  necessity,  produce  the  same  effect  as  disease  of  the  pyramidal 


SYMPTOMS.  255 

fibre  itself,  or  the  cell  in  the  cerebral  cortex  from  which  the  fibre 
Bprings.  We  shall  afterwards  see  that  this  consideration  has  very  im- 
portant applications. 

If  a  fibre  of  either  segment  is  seriously  damaged,  it  degenerates  below 
the  division,  since  its  nutrition  depends  on  the  influence  of  the  cell 
from  which  it  has  sprung.  If  damaged  only  by  pressure,  however 
complete  may  be  the  evidence  of  degeneration,  regeneration  is  possible, 
even  after  one  or  two  years.  It  is  possible  that  such  regeneration  also 
occurs  when  the  damage  is  by  inflammation.  The  nutritional  stability 
of  the  fibre,  or  rather  of  its  essential  element,  the  axis-cylinder, 
becomes  less,  and  more  easily  deranged,  the  greater  the  distance  from 
the  parent  cell,  and  it  is  least  in  the  terminal  ramification  of  each 
segment.  This  is  probably  the  reason  why  curara  acts  chiefly  on  the 
intra-muscular  nerves,  and  it  explains  the  influence  of  many  poisons 
on  the  peripheral  nerve-endings,  and  the  facts  of  nerve-degeneration 
in  tabes  and  multiple  neuritis,  &c.  The  law  is  probably  also  true 
of  the  upper  segment;  and  if  so,  many  facts  of  pathology  become 
clearer  to  us,  as  will  be  seen  in  the  chapter  on  spastic  paraplegia.  It 
may  thus  be  taken  as  a  general  law  of  the  utmost  importance  that  the 
vitality  of  the  processes  of  a  neuron  and  their  power  of  resisting  morbid 
influences  diminish  in  proportion  to  their  distance  from  the  cell  hody  of 
which  they  are  really  part. 

A  lesion  of  the  pyramidal  tract  causes  loss  of  power  in  all  parts 
below  the  level  of  the  disease — that  is  to  say,  of  all  parts  the  fibres  for 
which  are  interrupted.  A  lesion  of  the  grey  matter,  or  of  the  anterior 
roots,  causes  paralysis  only  of  those  parts  which  are  functionally  on 
the  level  of  the  lesion.  The  two  mechanisms  often  coincide.  A 
transverse  lesion  in  the  cervical  enlargement,  for  instance,  may  cause 
paralysis  of  the  arms  from  the  damage  to  the  grey  matter  and  nerve- 
roots,  and  paralysis  of  the  legs  from  damage  to  the  pyramidal  fibres. 
The  disease  involves,  primarily  in  the  former  case,  the  commencement 
of  the  second  segment  of  the  motor  path  for  the  arms  ;  in  the  latter, 
the  middle  of  the  upper  segment.  The  associations  of  the  paralysis 
differ  accordingly.  The  parts  affected  by  a  lesion  at  any  level  will  be 
readily  ascertaiaed  by  an  examination  of  the  table  of  functions  on 
p.  252. 

It  is  important  to  note  that  the  affection  of  motor  power  is  often 
incomplete.  It  may  then  involve  one  set  of  muscles  more  than 
another.  The  flexors  or  the  extensors  may  be  chiefly  paralysed.  In 
disease  of  the  dorsal  cord,  it  is  very  common  for  the  flexor  muscles 
of  the  hip  and  knee  to  suffer  more  than  the  extensors.  Why  this 
should  be  we  do  not  know,  but  the  fact  is  important,  because  con- 
siderable weakness  of  the  flexors  of  the  knee  is  readily  overlooked. 
In  examining  motor  power  each  set  of  muscles  should  be  separately 
tested. 

Motor  over-action ;  Spasm  is  frequent  in  disease  of  the  spinal  cord 
and  its  membranes.     It  may  present  the  form  of  tonic  spasm,  which 


256  SPINAL    CORD. 

when  persistent  is  termed  rigidity ;  or  of  clonic  spasm,  which  is  nsually 
transient  and  paroxysmal.  Tonic  spasm,  persistent,  and  involving 
only  a  certain  group  of  muscles,  causes  distortion  of  the  parts  to 
which  they  are  attached,  and  is  often  termed  contracture.'*  Tonic 
spasm,  paroxysmal  or  persistent,  is  far  more  frequent  than  clonic 
spasm,  which  occurs  chiefly  in  association  with  tonic  spasm  as  the 
muscular  clonus  described  at  p.  22,  or  as  a  consequence  of  certain 
functional  derangements.  Tonic  spasm  may  be  the  result  of  direct 
u-ritation  of  the  motor  structures,  or  may  depend  on  over-action  of 
the  reflex  centres,  due  not  to  irritation,  but  to  deficient  control. 
Either  form  may  be  acute  or  chronic.  The  acute  irritation  which 
causes  spasm  is  generally  inflammation  of  the  membranes,  rarely  an 
acute  lesion  of  the  substance  of  the  cord.  The  chronic  irritation  is 
chiefly  due  to  compression  of  the  motor  fibres — either  of  the  pyramidal 
tracts,  when  there  may  be  persistent  unvarying  spasms  in  the  parts 
below  the  disease,  related  to  the  fibres  irritated, — or  of  the  nerve- 
roots,  when  similar  spasm  is  produced  in  the  parts  at  the  level  of  the 
lesion.  The  spasm  that  depends  on  reflex  action  is  seen  chiefly  in  the 
legs  and  trunk,  but  especially  in  the  former.  It  may  preponderate 
in  either  the  flexors  or  extensors,  so  that  the  legs,  when  rigid,  may  be 
drawn  up  or  straight  out.  The  flexor  spasm  seems  to  be  due  to  an 
over-action,  of  the  centres  for  cutaneous  reflex  action,  the  extensor 
spasm  chiefly  to  that  of  the  centres  for  muscle-reflex  action,  although 
it  may  also  be  excited  indirectly  by  a  cutaneous  impression.  This  form 
will  be  considered  in  connection  with  the  reflex  over- action. 

Muscular  Contraction. — The  actual  shortening  of  muscles,  by  which 
they  cannot  be  passively  extended  to  their  normal  length,  is  due  to 
tissue  changes  fijxing  the  active  persistent  tonic  spasm  or  contracture 
just  mentioned.  The  rapidity  with  which  it  occurs  depends  on  the 
uniformity  of  the  spasm,  and  we  may  distinguish  three  modes  m 
which  it  arises,  which  differ  in  the  time  required  and  degree  attained. 
(1)  When  one  set  of  muscles  is  paralysed,  their  opponents,  aever 
being  extended,  quickly  become  fixed  in  the  contracted  condition 
which  they  assume  in  virtue  of  their  power  of  adaptation  to  posture. 
Thus  in  palsy  of  the  flexors  of  the  ankle,  the  calf  muscles  quickly 
become  contracted,  so  that  the  foot  cannot  be  flexed  on  the  leg  even 
up  to  a  right  angle.  The  same  thing  happens  by  a  similar  mechanism, 
when  one  posture  is  unceasingly  maintaiaed  and  one  set  of  muscles  is 
never  extended,  e.  g.  persistent  flexion  of  the  knee  leads  quickly  to 
structural  shortening  of  the  hamstring  muscles.  (2)  Where  there  is 
unchanging  active  contracture  from  chronic  irritation  of  the  motor 
fibres  by  a  growth,  or  permanent  reflex  contracture,  as  when  reflex 
flexor  spasm  keeps  the  hip  and  knee  permanently  flexed,  similar 
structural  changes  occur,  so  that  passive  elongation  soon  becomes 

*  The  reverse  of  this  condition,  loss  of  museular  tone  permitting  unusnal  freedom 
of  passive  movement,  is  described  in  tabes  under  the  name  of  hypotonie  musculaire 
(Frenkel). 


SYMPTOMS.  257 

impossible.  (3)  In  paroxysmal  tonic  spasm  of  intense  degree,  if  one 
Bet  of  muscles  is  stronger  tlian  their  opponents,  their  action  may  so 
preponderate  as  to  lead  to  a  greater  active  contracture  than  their 
opponents  present,  and  permanent  shortening  may  occur;  although  it 
does  so  slowly  and  to  a  slighter  degree  than  in  the  other  forms 
because  the  muscular  action  is  more  or  less  paroxysmal  and  varying, 
and  the  muscles  are  at  times  extended  by  their  opponents.  Thus  in 
paroxysmal  extensor  spasm  in  the  legs,  no  shortening  occurs  in  the 
muscles  moving  the  knee,  because  they  are  equally  balanced,  but  the 
calf  muscles,  being  stronger  than  the  flexors  of  the  ankle,  contract  the 
more,  and  extend  this  joint,  whenever  a  paroxysm  of  spasm  conies  on, 
and,  after  a  time,  may  undergo  slight  structural  shortening,  so  that 
the  foot  cannot  be  flexed  on  the  leg  beyond  a  right  angle. 

It  is  of  great  importance  to  distinguish  the  shortening  of  muscles 
due  to  tissue  changes  in  them,  from  the  active  contracture  that  may 
closely  simulate.it.  In  the  latter,  gentle  extension,  kept  up  for  a  few 
minutes,  restores  the  muscle  to  its  normal  length.  The  importance  of 
the  distiaction  is  due  to  the  fact  that  the  structural  shortening  can  be 
removed  by  tenotomy  or  forced  extension,  while  these  measures  are 
useless  in  the  case  of  active  contracture,  which  would  subsequently 
reassert  itself. 

Tremor  is  a  symptom  which  should  be  mentioned,  for  it  is  at  least 
associated  with  disease  of  the  spinal  cord  in  insular  sclerosis  (see  Vol. 
H).  It  also  occtirs  in  degenerative  diseases,  such  as  paralysis  agitans 
or  general  paralysis,  and  as  the  senile  tremor  of  old  age.  In  these 
conditions  it  probably  depends  upon  changes  in  the  cerebral  cortex.  It 
is  characteristically  present  in  toxic  conditions  manif  estiag  themselves 
chiefly  in  affections  of  the  peripheral  nerves,  such  as  those  arising  from 
alcohol,  lead,  and  mercury.  The  spontaneous  twitchings  of  muscular 
fibres  known  as  "  fibrillary  twitchings  "  occur  especially  in  association 
with  degenerative  disease  of  the  anterior  cornual  cells. 

Inco-ordination  of  movement,  although  motor,  depends  usually  on 
disease  of  afferent  fibres.  Its  characters  have  been  already  described 
in  outline  (p.  10). 

It  occurs  in  three  forms,  of  which  the  third  differs  entirely  from 
the  others  in  aspect  and  nature,  and  alone  is  a  true  motor  symptom. 
The  two  chief  forms  are  the  following: — (1)  Simple  disorder  of 
voluntary  musctdar  contractions,  which,  when  considerable,  amounts 
to  a  wild  irregularity  of  movement,  and  when  slight  is  only  manifested 
by  slight  irregularity  when  the  guidance  of  vision  is  withdrawn — as  in 
the  inability  to  maintain  the  muscular  contractions  in  the  perfect 
uniformity  needed  for  equilibrium  when  the  base  of  support  is  ren- 
dered small  by  the  juxtaposition  of  the  feet.  The  delicacy  of  the  test 
is  increased  by  the  removal  of  the  firm  base  afforded  by  the  boot,  and 
by  closure  of  the  eyes.  The  increase  in  unsteadiness  when  the  eyes 
are  closed  is  sometimes  termed  "Romberg's  symptom."  This  con- 
dition results  from  interruption  of  the  afferent  path  from  the  muscles 
VOL.  I,  17 


258  SPINAL    CORD. 

to  the  spinal  cord,  including  the  fibres  that  ascend  the  cord  ana  those 
that  subserve  the  muscle-reflex  process  at  the  level  of  entrance.  The 
effect  of  the  loss  of  the  latter  is  a  cessation  of  the  chief  part  of  spinal 
co-ordination,  which,  as  we  have  seen,  depends  on  those  processes, 
co-operating  with  the  voluntary  impulse.  Hence  it  is  associated  with 
loss  of  the  knee-jerk.  The  loss  of  other  forms  of  reflex  action  does 
not  seem  capable  of  causing  inco-ordination.  In  complete  interrup- 
tion of  the  root-fibres  from  the  muscles  the  mechanism  of  the  next 
form  is  necessarily  conjoined.  (2)  Inco-ordination  resembling  the 
slighter  degrees  of  that  just  described,  and  consisting  especially  in 
such  a  defect  of  equilibrium  as  results  from  disease  of  the  middle  lobe 
of  the  cerebellum,  is  produced  by  disease  of  the  posterior  median 
columns  above  the  lumbar  enlargement,  or,  if  it  extends  into  the 
latter,  leaving  the  root-zones  free  so  as  not  to  impair  muscle-reflex 
action.  This  is  apparently  produced  by  interru|.)tion  of  the  path  by 
which  impressions  from  the  muscles  are  conveyed  to  the  posterior  nuclei 
of  the  medulla,  and  probably  thence  to  the  cerebellum  (p.  244).  The 
effect  is  to  deprive  the  motor  cortex  of  the  guidance  afforded  by  these 
impulses.  The  difference  between  this  form  and  the  slighter  degrees 
of  the  first  is  that  the  muscle-reflex  action  is  not  lost,  the  knee-jerk 
being  preserved.  They  are  both  spoken  of  as  ataxy  also.  (3)  Irregular 
compression  of  the  motor  fibres  in  the  cord  apparently  causes  the 
peculiar  jerky  inco-ordination  seen  in  disseminated  sclerosis,  which  is 
supposed  to  depend  on  unequal  and  irregular  conduction  along  the  fibres, 
due  to  their  compression.     This  form,  however,  needs  further  study. 

Sensoky  Symptoms. — Loss  of  sensation  is  a  very  common  effect  of 
disease  of  the  spinal  cord.  It  may  be  total,  and  involve  all  forms  of 
sensibihty,  or  partial,  and  affect  only  some  forms.  The  statements 
made  on  pp.  13 — 19  regarding  impairment  of  sensation  generally,  and 
the  method  of  ascertaining  it,  apply  to  that  which  results  from  cord 
disease,  and  need  not  be  here  repeated.  It  has  been  also  pointed  out 
(p.  78)  that  loss  of  sensation  occurs  less  readily  than  loss  of  motor 
power.  In  cord  disease  it  is  common  for  muscular  paralysis  to  exist 
with  intact  sensibility.  This  may  partly  depend  on  the  fact,  ascer- 
tained by  experiments  on  the  effect  of  local  anaemia  of  the  cord,  that 
the  sensory  fibres,  of  the  nerve-roots  at  least,  seem  to  have  more 
resisting  power  than  the  motor  fibres.  Sensation  may  be  impaired  by 
disease  of  any  part  of  the  sensory  path-^posterior  roots,  probably 
also  the  posterior  cornua  and  commissure,  or  the  conducting  tracts  up 
the  cord.  A  division  of  the  sensory  path  into  upper  and  lower  seg- 
ments is  conceivable,  analogous  to  that  of  the  motor  path,  although 
we  have  not  the  same  clear  ground  for  the  distinction  into  simple 
segments  of  similar  composition.  Histological  researches  show  that 
most  of  the  centripetal  neurons  have  short  axis-cylinders,  and  suggest 
that  upward  conduction  is  subserved  by  a  number  of  relays. 

Disease  of  the  posterior  nerve-roots  causes  loss  of  reflex  action  as 
well  as  impairment  of  sensation,  just  as  disease  of  the  anterior  roots 


SYiMPTOMS.  259 

interferes  with  reflex  action  as  well  as  with  motor  power.  Internip* 
tion  of  the  sensory  conducting*  tracts  higher  up  leaves  reflex  action 
unaffected.  But  a  focal  lesion,  such  as  transverse  myelitis,  may 
damage  both  the  conducting  tracts  and  the  nerve-roots  at  the  level  of 
the  lesion,  or  may  affect  only  one  of  these.  Disease  outside  the  cord, 
compressing  it,  may  have  the  same  double  effect.  Hence  it  is  impor- 
tant to  test  sensation  at  the  level  of  the  lesion,  as  well  as  in  the  parts 
below,  and  to  remember  that  the  "  level  of  the  lesion  "  may  involve 
the  limbs  or  the  trunk.  Areas  of  anaesthesia  may  thus  be  found  on 
the  trunk  when  there  is  none  on  the  legs,  and  may  be  of  considerable 
diagnostic  importance,  e.g.  in  spinal  caries. 

Eandom  disease  of  the  nerve-roots  outside  the  cord  usually  impairs 
all  forms  of  sensibility,  although  slight  damage  may  arrest  the 
conduction  of  tactile  impressions,  and  not  those  of  pain,  which  are 
probably  more  energetic.  Disease  of  the  roots  within  the  cord  often 
causes  only  partial  loss,  because  the  fibres  which  conduct  different 
impressions  have  a  different  course.  But  loss  of  one  form  of  sensi- 
bility from  disease  of  the  root-fibres  or  nerve-fibres  usually  depends 
on  the  kind  of  disease,  and  is  especially  due  to  degenerative  changes, 
the  result  of  some  present  or  past  local  influence.  Disease  higher  up 
the  cord  still  more  frequently  causes  partial  loss ;  either  sensibility  to 
pain  or  to  touch  may  be  impaired.  That  to  temperature  is  rarely 
affected  without  that  to  pain.  We  do  not  yet  know  precisely  the 
significance  of  this  special  form  of  loss,  because,  as  we  have  seen,  we 
are  still  uncertain  as  to  the  path  for  each  form  of  sensibility.  It  is 
highly  probable,  however,  that  loss  of  sensibility  to  pain  is  produced 
by  disease  of  the  grey  matter  immediately  anterior  to  the  postero- 
median column. 

Disease  of  the  posterior  median  column,  and  possibly  that  of  the 
direct  cerebellar  tract,  certainly  impairs  the  conduction  of  impressions 
from  the  muscles.  It  probably  abohshes  the  "  muscular  sense "  of 
posture  and  movement,  although  this  does  not  entail  a  distinct  sen- 
sory loss.  But  there  may  be  also  a  greater  loss  of  muscular  sensibiliiy, 
so  that  the  normal  sensitiveness  to  pressure  and  passive  extension 
(and  even  at  last  to  electrical  stimulation)  may  be  impaired  or  lost. 
The  "  common  sensibility  "  of  muscles  is  probably  lost  only  when  the 
disease  of  the  nerves  is  greater  than  suffices  to  abohsh  what  is  termed 
the  "  muscular  sense."  Interference  with  this  path  in  the  nerves  or 
roots  outside  the  cord  abolishes  muscle-reflex  action,  and  also  causes 
more  pronounced  symptoms  (ataxy,  &c.)  than  disease  higher  up  the 
path  within  the  cord. 

Increased  sensitiveness,  hypersesthesia  and  hyperalgesia,  are  also 
common  in  disease  of  the  spinal  cord,  and  usually  depend  on  irritation 
of  the  conducting  fibres  in  some  part  of  their  course.  Probably  the 
irritation  produces  the  effect  by  intensifying  the  imjDulse  as  it  passes, 
since  the  phenomena  of  stimulation  of  nerves  show  that  their  axis- 
cylinders  have  some  power  of  transforming  other  forms  of  energy  into 


260  SPINAL    COBD. 

nerve-force,  i.e.  of  evolving  nerve-force,  and  therefore  of  increasing 
the  strength  of  that  which  passes  along  them. 

The  term  "  hyperaesthesia "  is  commonly  used  in  the  sense  of 
"hyperalgesia."  In  the  strict  sense  of  the  word,  hyperaesthesia  is 
seldom  observed  or  even  searched  for.  When  the  sensibility  to  touch 
is  "  increased  "  there  is  some  perversion  of  the  sensation,  not  a  simple 
increase.  The  sensation  may  be  felt  as  "thrilling"  or  "shock-like 
sensations."  When  there  is  an  extreme  increase,  pain  may  be  pro- 
duced by  a  touch,  but  it  is  more  probable  that  the  touch  stimulates 
the  over- sensitive  nerves  of  common  sensibility,  than  that  actual  pain 
is  produced  through  the  tactile  nerves.  A  touch  may  cause  pain  when 
it  js  not  felt  as  a  touch,  as  in  the  condition  termed  "anaesthesia  dolo- 
rosa." Delayed  sensibihty  to  pain,  or  temperature  especially,  is 
common  in  tabes,  and  is  occasionally  met  with  as  a  restdt  of  other 
changes  in  the  spinal  cord.  Impairment  of  sensibility  for  pain  and 
temperature  with  retention  of  ordinary  tactile  sensibility  is  charac- 
teristically met  with  in  syringomyelia.  Other  forms  of  altered  sensi- 
bility have  been  already  described. 

Ptim,  referred  to  the  spine,  occasionally  present  in  organic  disease 
of  the  cord,  is  more  frequent  in  disease  originating  in  the  meninges 
or  bones.  But  the  frequency  with  which  spinal  pain  is  present  in 
abdominal,  especially  gastric  disease,  and  in  neuralgic  affections, 
lessens  its  significance  when  it  exists  alone.  In  meningitis,  acute 
or  chronic,  and  in  meningeal  growths,  spinal  pain  is  frequent,  and  in 
organic  disease  of  the  bones  of  the  vertebral  column  it  is  an  almost 
constant  symptom,  and  is  combined  with  local  tenderness.  The  same 
combination  of  local  pain  and  tenderness  frequently  occurs  in  cases  of 
neiu'algic  pain,  "  rachialgia," — a  condition  that  is  often  loosely  termed 
"spinal  irritation,"  especially  when  it  succeeds,  as  it  often  does, 
concussion  of  the  spine.  Such  pain  is  usually  felt  through  a  consider- 
able extent  of  the  vertebral  column,  or  has  more  than  one  place  of 
chief  intensity  and  tenderness.  That  which  is  due  to  organic  disease 
is  usually  fixed  and  unchanging.  In  organic  disease  of  the  cord 
itself  pain  is  more  often  referred  to  the  neighbourhood  of  the  spine, 
to  the  loins  or  the  sacrum,  than  to  the  spinal  column  itself. 

Still  more  important  are  the  pains  that  are  referred  to  the  parts  to 
which  the  sensory  nerves  are  distributed,  and  have  hence  been  termed 
"  excentric "  or  "radiating"  paias.  They  are  of  two  kinds:  (1) 
those  due  to  the  irritation  of  the  posterior  nerve-roots  in  their  passage 
through  the  intervertebral  foramina,  through  the  membranes,  or 
through  the  posterior  colxmins  of  the  cord ;  (2)  those  produced  by 
irritation  of  the  sensory  conducting  tracts.  The  pains  of  the  first 
class  are  called  "  root-pains,"  and  are  often  intense.  They  correspond 
in  level  to  the  disease.  Those  of  the  second  class  are  sometimes 
acute,  especially  in  lesions  that  irritate  the  conducting  tracts  by  pres- 
sure, e.  g.  growths.  More  often  they  are  dull  pains,  closely  resembling 
rheumatism,  and  frequentlv  mistaken  for  rheumatism  by  the  patients 


SYMPTOMS.  261 

tliemselves  and  tlieir  medical  attendants.  The  mistalre  is  the  more 
easily  made,  because  other  symptoms  suggestive  of  spinal  disease  may 
be  inconspicuous,  and  the  rheumatoid  pains  in  chronic  cases  may 
be  influenced  by  weather,  being  mu^^h  more  troublesome  in  damp  and 
cold  than  in  fine  and  dry  weather.  In  all  cases,  persistent  rheumatic 
pains  in  the  limbs  should  suggest  the  possibility  of  spinal  disease,  and 
watch  should  be  kept  for  such  symptoms  as  local  loss  of  power,  or 
alterations  in  reflex  action.  There  is  a  third  class  of  pains— resem- 
bling root-pains — which  depend  on  degenerative  changes  in  the  nerve- 
fibres  ;  the  molecular  alterations  that  result  give  rise  to  upward 
impulses  of  considerable  intensity.  They  are  met  with  in  locomotor 
ataxy  and  in  multiple  neuritis,  and  may  be  dull  as  well  as  acute.  The 
position  in  which  these  various  radiating  pains  are  felt — legs,  trunk, 
or  arms — dep' nds  upon  the  seat  of  the  disease.  Although  often 
produced  in  the  root-fibres,  these  pains  may  also  be  due  to  the  peri- 
pheral nerves. 

It  is  especially  important  to  note  that  the  root-pains  are  frequently 
felt  as  a  sense  of  constriction,  a  painful  sense  of  tightness,  as  if  a 
band  were  tied  tightly  around  the  part — the  "girdle- pain,"  as  it  is 
called.  When  there  is  transverse  damage  to  the  cord,  at  the  lowest 
part  of  the  healthy  region  there  is  a  state  of  irritation  of  the  sensory 
nerves,  and  this  irritation  (referred  to  the  nerve-endings)  causes  the 
girdle-pain.  It  is  named  from  its  frequency  at  the  level  at  which  a 
girdle  is  worn,  the  middle  of  the  trunk,  which  depends  on  the  dorsal 
region  being  the  most  common  seat  of  disease;  but  it  may  be  felt 
lower  down,  about  the  groins  or  even  the  legs,  or  higher  up,  in  the 
arms.     In  the  limbs  it  is  a  mere  sense  of  tightness. 

The  girdle-pain  is  a  symptom  chiefly  of  inflammatory  and  degene- 
rative processes  within  the  cord.  Pressure  on  the  nerve-roots  usually 
causes  acute  pain,  but  the  most  severe  root-pains  are  those  met  with 
in  disease  of  the  bones  of  the  spine.  These  have  also,  more  than  any 
other  kind,  the  characteristic  that  they  are  increased  by  movement, 
and  in  growths  commencing  in  the  bones  (which  are  usually  malig- 
nant) this  feature  is  of  considerable  diagnostic  importance.  The 
suffering  in  such  cases  is  so  great  as  to  have  gained  for  the  resulting 
symptoms  the  ominous  designation  of  paraplegia  dolorosa.  The  pain 
in  this  condition  may  have  a  localising  value,  that  in  carcinoma  being 
frequently  referred  to  the  neighbourhood  of  the  column,  while  in 
caries  it  seems  more  frequently  to  affect  the  side  of  the  chest. 

Spontaneous  sensations,  other  than  pain,  are  very  common  in  disease 
of  the  spinal  cord,  and  are  often  of  considerable  diagnostic  im- 
portance, but  suggestive  rather  of  the  presence  of  a  morbid  state  than 
of  its  nattu-e.  They  may  present  many  varieties  of  character,  but  the 
familiar  sensation  produced  by  pressure  on  a  nerve-trunk,  when  the 
part  is  said  to  be  "asleep,"  or  to  have  "pins  and  needles,"  is  the 
most  common  and  the  most  significant.  It  may  occiir  in  functional 
disturbance  of  the  cord  as  -well  as  in  organic  disease,  and  also  is  very 


262  SPINAL    CORD. 

common  in  peripheral  nenritis.     It   depends  on  over-action  of  the 

sensory  structures,  but  it  has  at  present  no  special  significance  as  to 
the  locality  of  disease.  It  may  be  excited  by  contact  when  it  is  not 
spontaneous,  and  probably  represents  the  highest  degree  of  excess  of 
impressions  conveyed  by  the  nerves  of  tactile  sensibility.  The  still 
vaguer  feeling  called  "  numbness"  is  also  common,  and  may  occur,  in 
slight  degree,  in  diseases  of  which  all  other  symptoms  are  purely 
motor.  But  this  word  is  used  in  so  many  different  senses  that  the 
meaning  in  which  the  individual  uses  it  should  always  be  ascertained 
as  far  as  possible.  Sometimes  it  signifies  distinct  loss  of  sensibility  j 
more  often  a  feeling  as  if  there  ought  to  be  loss  when  there  is  not. 
Our  sensations  altogether  transcend  our  vocabulary,  but  the  observer 
must  try  (without  leading)  to  ascertain  the  character  of  the  sensation 
experienced. 

Eeflex  Action. — Loss  of  reflex  action  indicates  an  interruption  of 
the  reflex  arc  concerned.  This  interruption  may  be  anywhere  between 
the  peripheral  endings  of  the  motor  and  sensory  nerves,  and  thus  is 
not  necessarily  withm  the  spinal  cord,  or  even  within  the  spinal  canal, 
[t  is  as  constant  in  peripheral  neuritis  as  in  any  spinal  disease.  The 
position  of  the  interruption  must  be  determined  by  the  associated, 
symptoms  ;  if  it  is  in  the  centripetal  portion  of  the  reflex  arc,  there  ia 
impairment  of  sensation,  since  the  interruption  will  equally  arrest 
conduction  to  the  brain.  If  it  is  in  the  centrifugal  portion  of  the  arc, 
there  is  a  corresponding  interruption  in  the  path  of  the  voluntary  im- 
pulse, and  loss  of  motor  power.  Moreover  disease  of  the  motor  centre 
or  motor  nerves  causes  also  degeneration  of  the  nerves  and  wasting 
of  the  muscles.  Any  considerable  disease  of  the  nerves  abolishes  all 
reflex  action  from  the  part  they  supply,  but  disease  limited  to  the 
motor  structures  may  permit  a  reflex  movement  to  take  place  at  a 
distance,  although  preventing  it  at  the  part  stimidated. 

Disease  of  the  motor  centre  or  nerves  causes  loss  of  all  fonns  of 
reflex  action,  the  simple  form  and  the  muscle-reflex  that  underliea 
myotatic  irritability.  Partial  disease  of  the  afferent  path  may  impair 
one  and  not  the  other,  may  abolish  the  muscle-reflex  action  (myotatic 
irritability)  and  not  the  superficial  reflex  action,  since  the  afferent 
nerves  for  the  two  are  distinct,  coming  in  the  one  case  from  the 
muscles,  in  the  other  from  the  skin.  When  one  only  is  lost  it  is 
generally  the  muscle-reflex  action  ;  the  nerves  for  this  seem  more  sus- 
ceptible, and  to  have  less  power  of  resisting  morbid  influences. 

Loss  of  all  reflex  action  may  occur  as  a  transient  symptom,  imme- 
diately after  the  onset  of  an  acute  lesion  of  the  cord,  apparently  from 
irritative  inhibition  of  the  centres.  Cutaneous  reflex  action  may  be 
lessened  permanently  in  some  cases  of  brain  disease  on  the  side  of  the 
motor  palsy,  even  when  the  muscle-reflex  action  is  increased.  Indeed, 
this  opposite  change  in  the  two  forms  of  reflex  action  co-existing  on 
the  same  side  always  suggests  intra- cerebral  disease.  It  may  be  well 
again  to  remind  the  reader  how  difiicult  it  often  is  to  be  sure  whether 


SYMPTOMS.  263 

the  1< nee-jerk  is  present  or  is  lost,  on  accconnt  of  tlie  readiness  witli 
which  its  occurrence  may  be  prevented  by  inability  to  relax  the 
muscles,  and  its  presence  may  be  simulated  by  a  true  reflex  action 
(see  p.  238). 

Excess  of  reflex  action  implies,  of  necessity,  the  integrity  of  the 
reflex  arc  concerned,  and  shows  that  organic  disease,  if  it  exists,  is 
higher  up  the  cord.  Each  form  of  reflex  action  is  often  increased. 
In  some  acute  diseases,  as  acute  meningitis,  and  probably  also  in  some 
chronic  diseases,  the  increase  may  be  due  to  an  irritation  of  the 
centres,  but  in  most  forms  of  chronic  disease  it  is  apparently  the 
result  of  a  loss  of  control,  and  indicates  disease  between  the  centre 
concerned  and  the  brain.  We  have  already  considered  (p.  240)  the 
probable  mechanism,  and  have  seen  that  the  excess  of  the  muscle- 
reflex  action  is  related  to  disease  of  the  pyramidal  fibres,  and  espe- 
cially to  the  loss  of  the  terminal  part  of  these  fibres,  within  the  grey 
matter,  adjacent  to  the  centres  concerned.*  This  is  the  significance 
of  considerable  excess.  Degeneration  of  the  terminal  portions  of  the 
fibres  is  commonly  due  to  a  descending  degeneration  of  the  fibres  them- 
selves. It  is  possible,  however,  that  the  degeneration  of  the  endings 
of  the  pyramidal  fibres  may  be  primary,  as  is  that  of  the  nerve-fibres. 
(See  Primary  Spastic  Paraplegia.)  The  increase  of  reflex  action  is 
chiefly  manifested  in  the  legs,  in  which  reflex  action  is  normally  more 
active  and  important  than  in  the  arms.  The  increase  in  the  cutaneous 
reflex  action  is  shown  by  its  occurrence  on  a  shghter  stimulation  of 
the  stin,  and  by  a  greater  degree  and  extent  of  the  mxiscular  contrac- 
tions produced.  In  these  the  flexors  usually  predominate,  so  that  the 
leg  is  drawn  up  in  a  strong  movement.  As  the  action  of  the  sexual 
organs  depends,  in  part,  on  superficial  reflex  processes,  priapism  is 
common  when  the  reflex  excess  is  great.  The  increase  of  the  muscle- 
reflex  action  is  shown  by  an  increase  of  myotatic  irritability,  and 
therefore  of  the  so-called  "tendon-reflex  contractions," — the  increased 
knee-jerk,  rectus-clonus,  foot-clonus,  wrist-jerk,  elbow-jerk,  jaw-jerk, 
jaw-clonus,  &c.  These  have  been  already  described.  As  already 
stated,  the  excess  of  this  irritability  is  at  first  moderate,  and  slowly 
increases.  A  high  degree  of  over-action  is  never  suddenly  attained, 
as  it  may  be  in  the  case  of  cutaneous  reflex  action.  It  would  seem  as 
though,  in  the  centres  liberated  from  control,  a  capacity  for  excessive 
action  is  slowly  developed,  which  ultimately  results  in  tonic  spasm. 

Not  only  is  the  common  cause  of  this  excess  damage  to  the  pyra- 
midal trac+s  higher  up  the  cord,  entaihng  their  secondary  degenera- 
tion; but  if  such  disease  higher  up — as,  for  instance,  primary  myelitis 

*  It  is  interesting  to  note  the  .inalogy  between  the  effects  of  degeneration  of  the 
termination  of  the  two  segments  of  the  motor  path.  The  increased  activity  of  the 
muscle-reflex  I'entres,  which  results  from  degeneration  of  the  termination  of  the 
upper  segment,  presents  some  similarity  to  the  increased  voltaic  irritability  of  the 
muscular  fibres  which  results  from  degeneration  of  the  termination  of  the  lower 
seamen  D. 


264  SPINAL    COED. 

or  in  jury  to  the  cord — is  followed  by  abolition  of  the  muscle-reflex 
action  in  tbe  parts  below,  it  is  the  result  of  inflammatory  damage  to 
the  lower  part  of  the  cord  in  which  the  centres  are  situated,  generally 
by  a  descending  myelitis.  Sometimes  the  original  myelitis  involved 
the  whole  or  a  considerable  part  of  the  cord  below,  and  the  indications 
of  a  transverse  lesion  were  merely  those  of  the  upper  limit  of  the 
disease.  It  is  not  uncommon  for  the  secondary  myelitis  below  an 
original  transverse  lesion  to  succeed  it,  and  abolish  the  reflex  action 
which  was  at  first  unimpaired.  I  have  several  times  met  with  such  a 
sequence.  A  primary  dorsal  myelitis  has  left  reflex  action  in  the  legs 
unimpaired  and  tending  to  excess,  with  the  sphincter  ani  in  its  condi- 
tion of  uncontrolled  automatic  action,  when  after  a  few  days,  a  week 
or  more,  indications  of  a  recurrence  of  myelitis  have  been  attended  by 
abolition  of  all  reflex  action,  including  the  knee-jerk,  by  flaccidity  of 
the  muscles,  with  rapid  development  of  the  reaction  of  degeneration 
in  them,  and  by  complete  relaxation  of  the  sphincter  ani.  This  is  the 
state  from  the  first  in  the  cases  in  which  the  primary  myelitis  involves 
the  whole  lower  part  of  the  cord.  Sometimes,  indeed,  such  descend- 
ing inflammation  is  limited  to  the  grey  matter  or  to  the  posterior 
columns,  with  corresponding  limitation  of  the  symptoms,  but  with  a 
similar  abolition  of  reflex  action.  Of  course,  such  descending  inflam- 
mation is  a  very  differ,  it  thing  from  the  secondary  descending 
degeneration  of  the  pyramidal  tracts* 

Over-action  may  perhaps  be  also  produced  by  a  state  of  irritation 
propagated  from  above,  as  in  the  "  early  rigidity  "  of  hemiplegia ;  we 
have  seen  that  the  motor  impulses  probably  pass  from  the  pyramidal 
fibres  through  part  at  least  of  the  muscle-reflex  mechanism.  Primary 
over-action  seems  also  to  occur,  although  apparently  it  only  attains  a 
slight  degree.  It  is  probably  the  cause  of  the  trifling  excess  met  with 
in  cases  of  defective  nutrition  of  the  spinal  cord  or  of  the  nervous 
system  generally. 

Reflex  spasm. — We  have  seen  (p.  26)  that  the  muscular  state  on 
which  myotatic  irritability  depends,  assumed  to  be  due  to  a  muscle- 
reflex  action,  is  probably  identical  with  physiological  tone.  When- 
ever there  is  a  considerable  increase  in  the  irritability  there  is  a 
tendency  to  tonic  spasm,  in  which  there  is  a  balanced  contraction  of 
the  muscles,  fixing  the  limbs  in  the  position  of  extension.  At  first 
there  is  merely  slight  stiffness  of  the  legs,  especially  felt  on  waking 
from  sleep ;  afterwards  the  legs,  when  extended,  become  distinctly 
stiff,  although  they  may  be  quite  supple  when  flexed.     If  gradually 

*  In  consequence  of  the  occurrence  of  this  inflaramation  in  the  lower  part  of  the 
cord  having  been  overlooked,  it  has  been  thought  that  a  transvertie  lesion  higher  up 
has  the  power  of  abolishing  reflex  action  bel  i\v,  where  the  cord  is  not  damaged 
except  by  descending  degeneration.  See,  however,  p.  272,  note.  In  one  case,  how- 
ever,  described  by  Bruns  (*  Arch,  f .  Psychiatric,'  vol.  xxv),  the  knee-jerk  was  absent 
although  the  grey  matter  was  found  to  be  unaltered.  There  was  abolition  ol 
faradic  irritability  in  the  legs  ascribed  to  oedema. 


SYMPTOMS.  265 

extended,  when  near  full  extension  the  spasm  suddenly  comes  on  and 
completes  the  movement,  as  the  blade  of  a  pocket  knife  moves  under 
the  influence  of  the  spring.  Hence  this  has  been  termed  "  clasp- 
knife  rigidity."  Ultimately  the  extensor  spasm  may  be  so  great  that, 
when  it  occurs,  both  legs  are  rigid,  and  are  so  firmly  connected  with 
the  pelvis  that  if  one  is  lifted  from  the  bed  the  other  is  moved  also. 
This  is  only  met  with  in  organic  disease,  and  is  an  important 
diagnostic  indication.  Parox;ysms  of  spasm  may  be  brought  on  by 
any  impression  on  the  sensory  nerves,  a  prick  on  the  skin,  or  an 
attempt  to  obtain  the  clonus.  The  extreme  spasm  prevents  any 
clonus  being  obtained,  and  it  may  be  necessary  to  flex  the  limb  before 
an  attempt  to  obtain  it  is  successful.  As  violent  spasm  is  passing 
off,  moreover,  the  tension  of  the  muscles  may  alone  set  up  a  clonus, 
so  that  the  paroxysm  of  tonic  spasm  passes  into  clonic  spasm,  a 
phenomenon  that  was  termed  by  Brown-Sequard  "  spinal  epilepsy," 
on  account  of  the  superficial  resemblance  to  the  sequence  of  spasm  in 
an  epileptic  fit.  This  clonic  spasm  may  often  be  arrested  by  any 
strong  painful  impression  on  the  sensory  nerves,  a  pinch  of  the  skin, 
or  painful  flexion  of  the  big  toe ;  the  strong  stimulation  of  the  nerves 
of  common  sensation  inhibits  the  reflex  centre  concerned  in  the  clonus. 
There  is  always  much  weakness  of  the  legs  when  there  is  much  spasm, 
and  there  may  be  almost  complete  paralysis.  Hence  the  condition 
has  been  termed  "  spastic  paraplegia."  It  is  probable  that  the  central 
reflex  mechanisms  concerned  in  the  production  of  this  extensor  spasm 
are  those  that  subserve  the  act  of  standing.  The  spasm  often  enables 
a  paraplegic  patient  to  stand,  when  his  voluntary  power  is  quite 
insufficient  for  the  act.  In  advanced  cases  the  extensor  spasm  may  be 
varied  by  attacks  of  flexor  spasm,  which  at  first  come  on  chiefly 
during  sleep,  but  after  a  time  preponderate,  and  may  ultimately  be 
permanent ;  this  is  always  an  unfavorable  symptom,  since,  for  some 
reason  not  yet  understood,  this  flexor  spasm  indicates  a  condition  of 
the  spinal  cord  from  which  recovery  is  rare. 

Vaso-motoe  and  Trophic  Distttebance. — Changes  in  Nutrition. — 
Considerable  wasting  of  the  muscles  indicates  disease  of  the  motor 
tierve-cells,  or  of  the  nerve-fibres  pro.ceeding  from  these  cells.  If  the 
disease  is  acute  there  is  rapid  degeneration  of  the  nerves,  with  the 
reaction  of  degeneration  in  the  muscles  (see  p.  71).  The  wasting  in 
these  cases  is  always  great,  and  there  is  always  loss  of  reflex  action. 
Occasionally  some  fibres  of  the  nerve  and  muscle  suffer  and  others  do 
not,  causing  the  '•'  middle"  or  "mixed  "  form  of  reaction  (see  p.  32). 
In  rare  cases,  in  Avhich  the  nerve  degeneration  set  up  is  of  an  intensely 
irritative  character,  the  muscles  may  rapidly  lose  all  irritability  in 
<5onsequence  of  true  degeneration  of  the  fibres.  I  have  observed 
this,  for  instance,  in  a  growth  in  the  lumbar  region.  In  other  cases 
of  disease,  however,  there  is  a  slighter  degree  of  wasting  of  the 
muscles ;  there  is  no  loss  of  irritability  in  the  nerves ;  the  irrita- 
bility may  be  slightly  increased  or  lessened,  but  is  changed  in  the 


266  SPINAL    CORD. 

same  way  to  each  form  of  electricity.  In  this  condition  reflex  action 
is  not  lost,  but,  on  the  contrary,  is  often  increased.  The  change  is 
apparently  the  result  of  a  peculiar  alteration  in  the  nutrition  of  the 
nerve-cells.  It  is  usually  the  effect  of  an  irritative  degeneration  of  the 
pyramidal  fibres,  which  influences  in  a  peculiar  manner  the  nutrition 
of  the  motor  cells. 

The  changes  in  the  nutrition  of  the  skin,  if  slight  and  chronic, 
resemble  those  produced  by  disease  of  the  nerves  (see  p.  79),  and  are 
probably  produced  through  the  agency  of  the  posterior  roots.  Per- 
forating ulcer  and  the  trophic  disease  of  joint  known  as  "  Charcot's 
joint  "  are  met  with  especially  in  tabes.  They  also  occur  in  syringo- 
myelia. Sometimes,  however,  these  changes  are  most  acute,  Yery 
slight  pressure,  continued  for  a  few  hours,  causes  vesication,,  and  even 
sloughing  of  the  skin.  Sometimes  bullae  form  where  there  has  been 
no  pressure.  Occasionally  there  is  inflammation  of  the  joints.  Such 
intense  changes  are  only  met  with  when  the  morbid  process  in  the 
cord  is  irritative  in  its  character,  especially  in  haemorrhage,  and  in 
spreading  or  disseminated  myelitis.  The  points  on  which  sloughing 
occurs  with  greatest  readiness  are  the  heel,  over  the  malleoli,  the 
trochanters,  and  the  sacrum.  The  position  of  the  bedsores  in  para- 
plegia, usually  over  the  sacrum,  is  different  from  that  in  hemiplegia, 
generally  over  the  gluteal  region  on  the  paralysed  side.  The  tendency 
to  trophic  disturbance  probably  favours  the  occurrence  of  cystitis  from 
retention  of  urine,  and  the  readiness  with  which  the  inflammation 
invades  the  kidneys.  I  have  seen  a  low  form  of  suppurating  subperi- 
toneal pelvic  cellulitis,  in  a  case  of  disseminated  myelitis  with  cutaneous 
sloughs,  in  which  no  other  cause  for  the  celluHtis  could  be  found. 

Vaso-motor  disturbance  is  common  in  slight  degree,  irrespective  of 
the  seat  of  the  disease,  but,  like  the  trophic  changes,  which  probably 
depend  in  some  degree  upon  it,  it  is  always  most  intense  in  irritative 
lesions  of  the  cord.  The  slighter  and  common  degree  consists  merely 
in  increased  warmth  of  the  limbs,  in  which  the  vessels  dilate  too 
readily  ;•  while,  at  a  later  period,  the  limbs  are  colder,  paler,  and  often 
livid  at  the  extremities.  In  rare  cases  intense  flushing  of  the  skin  has 
been  observed,  or  copious  and  long-continued  pei-spirafcion.  When 
the  disease  is  in  the  lower  cervical  region,  flushing  and  perspiration 
may  occur  on  the  face  and  head. 

Disease  of  the  cervical  cord  or  nerves  is  sometimes  attended  by  a 
change  in  the  pupil,  on  one  side  only.  It  is  due  to  the  fact  that  the 
dilator  is  innervated  from  the  cervical  sympathetic  through  the  last 
cervical  nerves.  There  may  be  contraction,  from  paralysis  of  these 
radiating  fibres,  or  there  may  be  persistent  dilatation  from  their  irrita- 
tion. There  may  also  be  narrowing  or  enlargement  of  the  palpebral 
fissure  from  the  same  cause.  The  cause  of  the  double  myosis,  so 
common  in  some  degenerative  diseases  of  the  cord,  is  uncertain;  it  is 
associated  with  loss  of  the  light-reflex,  and  also  of  the  reflex  dilatation 
produced  by  stimulating  the  skin  of  the  neck. 


SYMPTOMS.  267 

The  frequency  of  the  heart's  action  is  often  permanently  increased 
in  locomotor  ataxy,  but  we  do  not  know  by  what  mechanism.  In 
disease  of  the  cervical  cord  (perhaps  also  in  that  of  the  cervical  nerve- 
roots)  the  heart  may  be  slowed  to  forty,  thirty,  or  twenty  beats  per 
minute,  usually  only  for  a  time.  This  is  frequently  met  with  in 
fracture  dislocation  in  this  region.  The  efi'ect  may  perhaps  be  due  to 
a  loss  of  the  influence  of  the  nerves  which,  when  stimulated,  quicken 
the  heart.  In  acute  disease  of  the  upper  dorsal  region  the  heart's 
action  may  be  persistently  frequent. 

Visceral  Disturhance. — Various  disturbances  in  the  alimentary  canal 
occur  in  disease  of  the  spinal  cord,  and  are  no  doubt  due  to  the  fact 
that  the  sympathetic  nerves  immediately  controlling  the  canal  are 
under  the  influence  of  the  cord,  and  may  be  excited  to  abnormal 
action  by  its  irritation,  or  may  underact  when  its  influence  is  lessened. 
Difficulty  of  deglutition  is  observed  in  some  cases  of  acute  disease  of 
the  upper  cervical  region.  Vomiting  occurs  also  in  disease  of  the 
same  part,  especially  in  fracture  of  the  cervical  vertebrae.  Constipa- 
tion is  extremely  common  in  disease  of  the  cord  above  the  lumbar 
enlargement.  The  "  gastric  and  laryngeal  crises  "  of  tabes  are  espe- 
cially frequent  in  cases  in  which  the  disease  is  situated  high  up  in  the 
cord.  When  rectum  and  bladder  are  affected,  the  disease  must  be 
lower. 

Paralysis  of  the  SpJiincters. — Incontinence  of  urine  and  f^ces  may 
be  due  to  disease  of  the  centres  that  control  the  action  of  the  spliinctei 
ani  and  the  muscular  mechanism  of  the  bladder  respectively,  or  it 
may  be  due  merely  to  disease  of  the  path  (probably  the  pyramidal 
tract)  through  which  voluntary  control  is  exerted.  In  the  latter  case 
the  sphincter  ani  acts  in  an  automatic  reflex  manner,  as  already  ex- 
plained. If  the  finger  is  introduced  within  it,  the  initial  relaxation  is 
felt  to  be  succeeded  by  a  firm  tonic  contraction.  This  proves  that  the 
anal  centre  in  the  lumbar  enlargement,  and  the  nerves  proceeding  from 
it,  are  intact.  If  this  centre  is  destroyed,  or  the  nerves  interrupted, 
no  tonic  contraction  can  be  felt;  there  is  complete  and  persistent 
relaxation.  I  have  .more  than  once  observed  a  dorsal  transverse 
myelitis  arrest  the  voluntary  influence  and  leave  the  sphincter  in 
its  condition  of  simple  reflex  action,  and,  a  week  or  ten  days  later,  the 
extension  of  the  myelitis  into  the  lumbar  region,  to  be  attended  by 
(among  other  signs)  perfect  inaction  of  the  sphincter.  A  rectal 
examination  will  then  often  give  important  information  regarding  the 
extent  of  the  disease  downwards. 

The  sphincter  of  the  bladder  is  not  accessible  to  such  direct 
examination,  and  the  mechanism  seems  to  be  more  complex,  but  we 
may  distinguish  conditions  corresponding  to  those  of  the  rectum.  If 
the  lumbar  centre  is  destroyed  the  sphincter  is  permanently  relaxed  j 
urine  dribbles  out  of  the  bladder  as  fast  as  it  enters  it;  there  is- 
simple  incontinence.  If  there  is  an  interruption  of  the  voluntary- 
path  above  the  centre,  the  sphincter  may  act  automatically :  when  a 


^Q8  SPINAL    CORD, 

certain  amount  of  urine  has  collected  in  the  bladder  it  excites  the 
muscular  fibres  in  the  wall  to  contract ;  the  sphincter  relaxes,  and  the 
urine  escapes, — there  is  intermittent  incontinence.  In  this  condition 
the  mechanism  is  excited  to  action  by  any  pressure  on  the  bladder ; 
a  movement,  or  the  act  of  coughing,  will  make  the  urine  come  away. 
But  the  muscle  of  the  wall  of  the  bladder  is  indirectly  under  the 
influence  of  the  will ;  when  the  sphincter  is  voluntarily  relaxed  the 
detrusor  fibres  in  the  wall  contract.  Loss  of  voluntary  power  seems 
to  lead  to  weakness  in  the  wall  of  the  bladder,  so  that  the  bladder  is 
never  perfectly  emptied,  and  ultimately  the  detrusor  cannot  contract 
to  expel  the  urine,  e^an  when  the  sphincter  is  relaxed.  Hence  reten- 
tion of  urine  occurs.  When  a  certain  degree  of  distension  of  the 
bladder  is  attained,  the  pressure  becomes  sufficient  to  force  the  urine 
out  whenever  the  sphincter  relaxes,  and  such  relaxation  is  continually 
occurring  under  the  high  pressure  that  is  repeatedly  renewed  in 
consequence  of  the  flow  from  the  kidneys.  Hence  incontinence 
results, — "overflow  incontinence"  (see  p.  247).  The  retention  often 
develops  very  gradually  and  insidiously  when  the  voluntary  power  is 
only  lessened ;  the  residual  urine  is  at  first  small  in  amount,  but 
gradually  increases  as  the  detrusor  fails  to  contract  adequately  against 
the  resistance.  It  is  important,  if  the  urine  comes  away  involuntarily, 
to  ascertain  which  form  of  incontinence  exists ;  since  overflow  incon- 
tinence, and  the  retention  it  indicates,  have  much  graver  consequences 
than  simple  incontinence.  The  permanent  distension  of  the  bladder 
constitutes  a  permanent  hindrance  to  the  flow  of  urine  from  the 
kidneys,  and  serious  kidney  disease  may  result.  This  is  a  not  uncom- 
mon cause  of  death  in  spinal  disease.  Moreover,  whenever  the 
bladder  is  habitually  emptied  imperfectly,  decomposition  is  apt  to 
occur  in  the  residual  urine.  The  decomposition  is  often  aided  by  the 
lessened  acidity,  or  even  actual  alkalinity,  of  the  urine.  This  change 
in  the  composition  of  the  urine  excites  inflammation  of  the  bladder ; 
pus  is  formed  by  the  mucous  membrane,  and  this,  in  its  turn, 
increases  the  decomposition  of  the  urine.  The  damage  to  the  kidneys, 
by  the  hindrance  to  the  flow  of  urine  from  them,  may  be  increased  by 
inflammation  ascending  the  ureters  from  the  bladder. 

Pyrexia. — The  initial  elevation  of  temperature  in  some  spinal 
lesions  may  be  due  to  them  directly,  but  sometimes  it  is  due  to  a 
blood- state  of  which  the  cord  disease  is  a  result.  This  is  probably  the 
case  in  polio-myelitis.  The  temperature  is  sometimes  raised  in  dis- 
ease of  the  cord  apart  from  the  influence  of  morbid  process.  Disease 
of  the  upper  cervical  cord,  like  that  of  the  medulla,  may  cause  hyper- 
pyrexia, and  this  may  also  result  from  acute  central  lesions  that 
occupy  a  considerable  vertical  extent,  such  as  a  central  haemorrhage, 
although  by  what  mechanism  these  act  we  do  not  know.  Slight 
variable  elevation  of  temperature  sometimes  occurs  in  disease  of  the 
dorsal  cord,  and  is  probably  due,  in  some  way,  to  the  disturbance  of 
its  functions  rather  than  to  any  influence  of  the  morbid  process.     For 


SYMPTOMS.  26^ 

instance,  in  one  case  of  hsemorrhagic  myelitis  witli  damage  from  the 
third  dorsal  to  the  second  lumbar  segments,  every  application  of 
electricity  to  the  legs  was  followed  by  slight  pyrexia. 

Convulsions. — Lastly,  convulsive  attacks  have  been  known  to  attend 
the  onset  of  acute  lesions  in  various  parts  of  the  cord,  in  adults  as 
well  as  in  children.  They  are  most  frequent  in  disease  of  the  cervical 
cord,  but  I  have  known  a  convulsion  to  occur  in  an  adult  at  the  onset 
of  myelitis  in  the  lower  dorsal  region.  The  convulsions  are  general. 
Their  mechanism  is  unknown,  and  in  some  cases,  especially  of  infan- 
tile polio -myelitis,  they  may,  like  the  initial  pyrexia,  be  the  result  of 
general  disturbance  of  the  system  by  an  acute  morbid  blood-state. 

Optic  neuritis  has  been  met  with,  especially  in  connection  with 
disease  in  the  cervical  region  of  the  cord,  and  in  cases  in  which  careful 
examination  of  the  brain  post  mortem  failed  to  reveal  any  cerebral 
disease. 

Combination  of  Symptoms. — Certain  symptoms  are  frequently 
combined,  and  their  combination  has  a  definite  localising  significance. 
Loss  of  motor  power  occurs  in  two  different  associations,  according  to 
the  position  of  its  cause  in  the  motor  path.  It  may  be  combined 
with  muscular  wasting,  loss  of  electric  irritability,  and  loss  of  all 
reflex  action.  In  this  case  the  disease  is  in  the  anterior  cornua  or 
nerve-roots,  the  lower  segment  of  the  motor  path.  (But  the  same 
symptoms  are  produced  by  disease  of  the  nerves  themselves — in 
multiple  neuritis.)  On  the  other  hand,  there  may  be  no  wasting,  no 
change  in  irritability,  and,  instead  of  a  loss  of  reflex  action,  the  myo- 
tatic  irritability  is  increased.  The  interruption  of  the  motor  path  is 
then  in  the  upper  segment,  usually  in  the  pyramidal  tracts.  It  may 
be  a  focal  lesion  of  the  cord,  or  a  primary  degeneration  of  the  tracts. 
The  distinction  is  that,  in  the  latter  case,  there  is  nothing  more  than 
the  muscular  weakness  and  increased  myotatic  irritability.  In  the 
former  case  there  are,  or  have  been,  indications  that  the  lesion  has 
extended  beyond  the  purely  motor  elements. 

The  symptoms  caused  by  disease  of  the  several  elements  of  the  cord 
have  now  been  described,  but  it  is  necessary  to  consider  further  the 
combination  of  symptoms  that  results  from  a  total  transverse  lesion  of 
the  cord,  and  from  a  unilateral  lesion. 

A  total  transverse  lesion,  however  limited  in  vertical  extent,  sepa- 
rates from  the  brain  all  parts  below  it,  and  hence,  so  far  as  will  and 
feehng  are  concerned,  it  produces  the  same  effect  as  if  the  whole  of 
the  cord  below  the  lesion  were  destroyed.  Compression  of  the  cord  in 
the  middle  of  the  cervical  enlargement,  for  instance,  aboHshes  motion 
and  sensation  in  all  parts  below  the  distribution  of  the  cervical  plexus. 
A  transverse  lesion  at  the  second  dorsal  nerve  causes  motor  and 
sensory  palsy  of  the  trunk  and  legs,  leaving  the  arms  unaffected.  But 
the  same  loss,  motor  and  sensory,  results  from  disease  which  damages 
the  whole  cord  up  to  the  level  indicated.  Hence  the  extent  of  the 
motor  and  sensory  paralysis  indicates  only  the  upward  limit  of  the 


270  SFliNAL    CORD. 

lesion;  how  far  it  extends  downwards  is  shown  by  the  interference 
with  the  central  and  especially  the  reflex  functions  of  the  cord.  The 
upward  level  is  indicated  not  only  by  the  loss  of  conduction,  but  also 
by  the  position  of  the  girdle-pain  and  radiating  pains,  or  zone  of 
hypersesthesia,  which  are  due  to  the  irritation  of  the  lowest  sensory 
nerves  in  the  upper  portion. 

It  is  desirable  to  know  the  symptoms  of  impaired  concliiction  that 
occur  when  a  transverse  lesion  is  at  different  levels  in  the  spinal  cord. 
They  may  readily  be  ascertained  by  an  examination  of  the  table  and 
figures  given  at  pp.  250 — 252.  There  is,  however,  some  uncertainty  as 
to  the  effect  on  sensation  in  the  limbs  produced  by  disease  at  different 
parts  of  the  lumbar  and  cervical  enlargements,  since,  as  we  have  seen, 
the  exact  representation  of  sensation  in  the  cord  is  still  uncertain. 

The  upper  limit  of  the  lesion  is  shown  by  the  upward  extent  of  the 
motor  and  sensory  loss.     The  lowest  nerves  supjjly  the  anus  and  peri- 
nseum.     Those  that  supj^ly  the  sMn  and  muscles  of  the  leg  and  foot 
arise  from  the  fourth  lumbar  to  the  second  sacral  segments,  and  are 
damaged  by  a  lesion  involving  the  lower  part  of  the  lumbar  enlarge- 
ment.    In  the  middle  of  the  lumbar  enlargement  the  nerves  arise 
which  enter  the  lumbo-sacral  cord,  and  these  are  probably  destined 
for  the  flexors  of  the  knee,  and  for  the  hip  muscles  supplied  by  the 
sacral  plexus,  the  glutei,  quadratus,  and  gemelli,  and  for  the  skin  of 
the  lower  part  of  the  gluteal  region.     These  parts  then  will  be  para- 
lysed by  disease  in  the  middle  of  the  lumbar  enlargement,  while 
the  muscles  and  skin  in  front  of  the  thigh  are  unaffected.      The 
latter  suffer  when  the  disease  affects  the  upper  part  of  the  lumbar 
enlargement,  the  origin  of  the  anterior   crural    (rectus,   &c.),   and 
obturator  (adductors).     The  skin  on  the  upper  and  outer  parts  of 
the  thigh  loses  sensibility,  with  the  part  adjacent  to  the  scrotum, 
and  in  the  groin,  only  when  the  disease  damages  the  highest  part 
of  the  lumbar  enlargement,  from  which  the  second  and  third  lumbar 
nerves  arise,  and  then  the  flexors  of  the  hip  become  paralysed.     In 
proportion  as  the  disease  is  higher  in  the  dorsal  region  we  have  the 
symptoms  ascending  higher  up  the  trunk,  and  marking  accurately  the 
height  of  the  lesion  by  the  loss  of  cutaneous  sensibility,  and  by  the 
impairment,   first  of  the  abdominal  muscles,   and  then   of  the   in- 
tercostal muscles.     The  umbilicus  corresponds  to  the  tenth   dorsal 
nerves,  and  the  ensiform  area  to  the  sixth  and  seventh.     When  the 
disease  reaches  the  lowest  part  of  the  cervical  enlargement  (the  first 
dorsal  nerves)   we  have  the  first  symptoms  in  the  upper  extremity ; 
but  these  are  not,  as  might  be  expected,  in  the  muscles  moving  the 
shoulder-joint,  but  in  the  hand.     The  first  numbness  is  complained  of 
in  the  little  finger,  and  the  first  weakness  is  in  the  intrinsic  muscles  of 
the  hand.     Ascending  higher,  the  symptoms  pass  up  the  arm  with 
some  uniformity,  and  without  respect  to  nerve  distribution.     When 
the  middle  of  the  cervical  enlargement  is  reached  (the  fifth,  sixth,  and 
seventh  cervical)  the  shoulder  muscles  and  the  serratus  magnus  be- 


SYMPTOMS.  271 

€ome  paralysed,  and  there  is  general  loss  of  power  and  sensation.  (Tot 
details  see  table,  p.  252,  and  also  pp.  250  and  251.)  AboTe  the  level  ol 
the  sixth  pair  the  trapezius  and  stemo-mastoid  become  somewhat 
weakened,  for  the  fibres  of  the  spinal  accessory  which  supply  them  un- 
doubtedly arise  in  part  from  this  region  of  the  cord.  At  the  fourth 
and  fifth  cervical  the  lower  part  of  the  neck  becomes  anaesthetic,  and 
the  diaphi-agm  ceases  to  act.  Here  our  localisation  might  cease,  for 
total  transverse  lesions  at  this  spot  necessarily  cause  death.  For  a 
little  time  the  sterno-mastoids  and  scaleni  can  stiU  get  some  air  into 
the  chest,  rarely  in  sufficient  amount  to  maintain  life  for  more  than  a 
few  days.  But  limited  lesions  may  occur  higher  up,  and  then  we  have 
complete  powerlessness  of  the  muscles  moving  the  head,  upper  part  of 
trapezius  and  sterno -mastoid,  and  other  muscles  attached  to  the 
occipital  bone,  and  interference  with  sensation  in  the  neck  and  parts 
of  the  head,  which  are  not  supplied  by  the  fifth  nerve. 

The  extent  downwards  of  the  lesion,  its  vertical  extent,  is  thus  not 
indicated  by  the  impairment  of  the  conducting  functions,  by  the  motor 
or  sensory  paralysis ;  to  ascertain  it  we  have  to  examine  the  functions 
of  the  cord  as  a  central  organ,  and  to  ascertain  how  far  they  are 
impaired  in  the  paralysed  region — to  examine  especially  muscular 
nutrition  and  reflex  action.  The  state  of  muscular  nutrition  and 
irritability  indicates  how  far  the  anterior  cornua  are  injured.  The 
relation  of  the  several  groaps  of  muscles  to  the  cord  is  shown  in  the 
first  column  of  the  table.  The  integrity  of  reflex  action  indicates  the 
integrity  of  the  reflex  loops,  and  the  study  of  the  superficial  reflexes  of 
the  trunk  is  especially  instructive  in  this  respect.  The  series  of  reflexes, 
and  the  relation  of  each  to  the  cord,  are  shown  in  the  second  column 
of  the  table ;  the  muscle-reflex  contractions  are  piinted  in  italics  in  the 
position  which  corresponds  to  the  centres  and  nerve-roots  which  are 
essential  for  their  production.  Excess  of  superficial  reflex  action  indi- 
cates loss  of  the  cerebral  control  exerted  on  the  reflex  centres,  and  con- 
siderable excess  of  the  muscle-reflexes  implies  impaired  function  of  the 
lowest  part  of  the  pyramidal  tracts,  and  generally  shows  the  existence 
of  a  descending  degeneration  in.  the  lateral  columns.  Keflex  action 
in  the  legs  may  be  abolished  for  a  few  hours  by  acute  disease  or 
injury  above  the  lumbar  enlargement,  ia  consequence  of  inhibitoiy 
shock,  but  soon  returns  if  the  lumbar  centres  are  intact.  As  already 
explained  (p.  264),  if  there  is  no  return  of  reflex  action  or  myotatic 
irritability,  their  absence  shows  that  the  lumbar  region  is  involved  in 
the  primary  lesion  ;  secondary  extension  to  this  is  indicated  by  loss  of 
reflex  action  distinctly  subsequent  to  the  onset.  If  such  loss  occurs 
vdthout  wasting  and  loss  of  faradic  iiritability  in  the  muscles,  it 
shows  that  the  disease  in  the  lumbar  region  is  limited  to  the  posterior 
columns  or  cornua.* 

•  A  few  cases  have  been  met  with  in  which  a  transverse  lesion  of  the  dorsal 
cord,  esp'i'ially  a  transvi-rse  concussion- myelitis,  without  post-mortem  signs  of 
lumbar  inllainmation,  has  caused  a  persistent  loss  of  reflex  aetJon  m  the  legs.     In 


272  SPIJSIAL    CORD. 

The  fact  of  chief  importance  is  that  dorsal  cord  disease,  the  most 
common  form,  causes  no  persistent  loss  of  reflex  action  in  the  legs, 
and  that  for  such  loss  special  causes  must  be  sought. 

Unilateral  lesions  interrupt  the  motor  path  to  the  muscles  on  the 
same  side  as  the  lesion,  causing  one-sided  palsy,  termed  "  spinal  hemi- 
plegia "  when  the  disease  is  so  high  as  to  affect  both  arm  and  leg, 
"  hemiparaplegia  "  when  it  is  lower,  and  affects  one  leg  only.  There 
is  often  some  loss  of  power  on  the  opposite  side,  which  may  be  due 
either  to  slighter  damage  to  the  other  side  of  the  cord  (since  few 
lesions  are  strictly  unilateral)  or  to  damage  to  non-decussating  fibres. 
Conversely,  the  paralysis  of  the  leg  may  be  incomplete  when  that  of  the 
arm  is  complete,  owing  to  the  escape  of  the  fibres  for  the  leg  which 
cross  lower  down  the  cord.  Sensation  is  affected  on  the  opposite  side, 
but  not  quite  up  to  the  level  of  the  lesion,  because  the  decussation  of 
the  sensory  tract  is  not  immediate,  but  occurs  somewhat  above  the 
entrance  of  the  nerves.  The  upper  level  may  vary  for  different  forms 
of  sensibility,  in  consequence  probably  of  the  level  of  crossing  (in  rela- 
tion to  entrance)  being  different  for  the  several  paths.  A  lesion  in 
one  side  of  the  lumbar  enlargement  often  affects  sensation  on  the 
same  side  as  motion,  because  it  damages  the  sensory  path  before  it 
has  crossed.  In  all  cases  of  crossed  motor  and  sensory  paralysis  the 
sensibility  of  the  muscles  differs  from  the  other  forms  of  sensibility ; 
and  if  it  is  affected  on  one  side,  this  is  the  side  of  the  motor  palsy,  and 
not  of  the  cutaneous  ansesthesia.* 

The  crossed  affection  of  cutaneous  sensibility  may  involve  aU  forms 
of  sensation  or  only  some  of  them.     Sensibility  to  pain  is  almost  in- 

these  cases,  however,  there  has  been  not  only  loss  of  the  muscle-reflex  action,  but  also 
of  the  skin  reflexes,  a  clear  indication  of  an  exceptional  condition.  Moreover  there  has 
also  in  other  cases  been  loss  of  faradic  irritability  and  rapid  muscular  wastino:,^in 
short,  there  have  been  all  the  indications  of  a  lumbar  myelitis.  The  most  probable 
explanation  of  these  cases  is  that  the  descending  degeneration  of  the  pyramidal 
tract  has  been  more  than  usually  irritative  in  nature,  so  as  practically  to  amount  to 
a  parenchymatous  inflammation,  and  that  this  has  invaded  the  motor  structures  as 
a  nutritional  change,  sufficient  to  abolish  their  function,  without,  however,  destroy- 
ing their  form.  In  one  such  case  it  was  noted  that  the  lumbar  nerve  elements 
were  extremely  granular  in  aspect.  For  a  different  explanation  of  these  cases  see 
Bastian,  'Med.-Chir.  Trans.,' 1890;  Bruns,  '  Neurol.  Centralb.,'  1895,  and  •  Arch, 
f.  Psych.,*  XXV ;  and  Egger,  '  Arch.  f.  Psychiat.,*  xxvii ;  see  also  note,  p.  264. 

*  This  was  first  pointed  out  by  Brown-Sequard  in  his  important  study  of  these 
cases.  Scepticism  has  been  expressed  as  to  tlie  fact,  based  on  a  denial  that  the 
test  commonly  employed,  recognition  of  posture,  is  significant.  Experiments  on 
animals,  even  monkeys,  are  inconclusive,  but  the  evidence  from  cases  in  man  is  very 
strong,  not  only  of  the  occurrence  of  the  loss,  but  of  the  significance  of  the  test.  In 
some  cases,  moreover,  the  muscles  were  insensitive  to  pressure.  One  recorded 
instance  is  the  following  : — A  stab  in  the  dorsal  region  caused  loss  of  cutaneous  sen- 
sibility in  the  right  leg  and  motor  palsy  in  the  left.  On  the  left  side,  on  which 
cutaneous  sensibility  was  normal,  the  sense  of  posture  was  absolutely  lost,  while  it 
was  present  on  the  right  side,  on  which  cutaneous  sensibility  was  impaired.  The 
patient  often  thought  the  left  leg  was  flexed  when  it  was  extended.  Ultimately  this 
leg  presented  distinct  ataxy.     (Gilbert,  'Arch,  de  Nevrologie,'  1882,  p.  275.) 


SYMPTOMS. 


273 


variably  impaired.  The  temperature  sense  is  usually  affected  witli 
that  for  pain;  in  only  two  of  twenty  recorded  cases  (in  which  the 
affection  of  sensibility  was  carefully  noted)  was  the  sense  of  tempera- 
ture normal,  and  that  of  pain  impaired,  and  in  neither  of  these  cases 
was  the  sensibility  to  pain  actually  lost.  On  the  other  hand,  in  one 
,  third  of  the  cases  tactile  sensibility  was  unaffected,  and  in  about  one 
tenth  of  the  cases  it  was  impaired  on  both  sides. 

Cutaneous  sensibility  is  sometimes  impaired  in  a  zone  at  the  level 
of  the  lesion,  and  on  the  same  side,  in  consequence  of  the  damage  to 
the  nerve-roots  entering  the  cord,  and  above  this  there  may  be  a 
narrow  band  of  hypersesthesia  from  irritation  of  the  roots  at  the 
upper  part  of  the  lesion.  On  the  side  of  the  lesion,  below  the  anaes- 
thetic zone,  there  is,  in  most  cases,  a  remarkable  hypersesthesia,  corre- 
sponding in  distribution  with  the  anaesthesia  on  the  opposite  side. 
Painful  impressions  are  felt  most  acutely,  and  even  a  touch  on  the 
sMn,  or  warm  or  cold  bodies,  produce  pain.  The  cause  of  this 
hypersesthesia  is  obscure.  Both  it  and  the  opposite  loss  of  sensibility 
may  last  for  twenty  years,  and  it  cannot  be  ascribed  therefore  to  any 
irritation  by  the  morbid  process.  It  is  perhaps  due  to  an  altered 
action  of  the  cerebral  centres  on  the  opposite  side  of  the  brain. 
There  is  much  evidence  of  an  intimate  connection  between  the  sensory 
centres  on  the  two  sides,  and  it  is  conceivable  that  the  altered  func- 
tional state  of  the  centre  pn  the  side  of  the  lesion,  to  which  impressions 
cease  to  come,  may  induce  in  the  opposite  hemisphere  a  condition 
expressed  as  hypersesthesia.  The  condition  may  be  thought  of  in 
relation  to  the  peculiar  phenomena  of  transfer  in  hysterical  hemi- 
ansesthesia. 

Eeflex  action  is  increased  in  all  its  forms  on  the  side  of  the  lesion, 
but  the  increase  only  occurs  after  some  days.  At  first  it  is  lessened  or 
even  abolished,  no  doubt  from  the  inhibitory  influence  of  the  irritation 
of  the  morbid  process. 

The  side  below  the  lesion  is  at  first,  for  some  days  or  even  weeks, 
warmer  than  the  other,  the  difference  being  usually  about  a  degree 
Fahrenheit.  In  the  course  of  time  this  difference  passes  away,  and 
the  side  may  even  be  colder  than  the  other. 

These  symptoms  may  be  shown  in  the  form  of  a  table : 


Zone  of  cut.  hypersesthesia. 
-^— — —  ansesthesia. 


Motor  palsy. 
Hypersesthesia  of  skin. 
Muscular  sense  impaired. 
Bi^flex  action  first  lessened,  then 

increased. 
Temperature  raised. 


VOL.  I. 


LESION. 


I 


Muscular  power  nornnal. 
Loss  of  sensibility  of  skin. 
Muscular  sense  normal. 
Beflex  action  normal. 

Temperature  same  as  that 
above  lesion. 


18 


274  SPINAL   COED. 

The  vertical  distribution  of  tlie  symptoms  varies  according  to  the 
vertical  position  of  the  lesion.  Their  relations  are  best  marked  when 
the  lesion  is  in  the  dorsal  region  of  the  spinal  cord.  When  it  is  in 
the  cervical  or  lumbar  enlargement  the  upper  limit  is  less  distinct 
because  of  the  complex  representation  of  skin  and  muscle  in  the 
nerve-roots. 


GENEBAL  PATEOLOQT AND  ETIOLOGY:  INDICATIONS  OF  THE 
NATURE  OF  TEE  LESION:   FATSOLOgHCAL  DIAGNOSIS. 

The  kinds  of  disease  to  which  the  spinal  cord  is  liable  are  not 
specifically  numerous,  but  those  which  are  common  present  several 
varieties.  The  processes  are  congestion  and  anaemia,  haemorrhage, 
inflammation,  degeneration,  and  growths.  Haemorrhage  and  growths 
are,  on  the  whole,  rare.  Congestion  plays  an  uncertain  part  in  the 
production  of  symptoms  ;  its  influence  has  been  certainly  overrated 
by  some  writers,  and  perhaps  under-estimated  by  others.  Anaemia 
is  now  known  to  be  sometimes  associated  with  certain  definite 
changes  in  the  spinal  cord,  and  it  is  possible  that  it  may  occa- 
sionally give  rise  to  symptoms  without  producing  gross  disease.  The 
most  frequent  lesions  are  inflammation  and  degeneration.  These  two 
processes,  distinct  in  their  typical  forms,  blend  in  their  intermediate 
varieties.  Inflammation  varies  much  in  character  and  course.  Its 
effects  are  in  some  cases  produced  with  great  rapidity,  in  other  cases 
with  extreme  slowness.  It  probably  commences  in  most  instances 
outside  the  nerve-elements,  which  are  secondarily  involved.  Perhaps 
some  acute  processes  in  the  nerve-elements  themselves  are  most  accu- 
rately regarded  as  a  parenchymatous  inflammation,  analogous  to  that 
which  we  know  occurs  in  the  fibres  of  the  peripheral  nerves.  Degene- 
ration consists  of  two  processes,  a  wasting  of  the  nerve-structures,  cells 
and  fibres,  and  an  overgrowth  of  the  connective-tissue  elements.  The 
latter  leads  to  the  condition  termed  sclerosis.  The  name  has  been 
given  to  it,  apparently,  because  the  process  is  one  that,  in  other 
organs,  increases  their  consistence.  In  the  cord  it  has  not  often  this 
effect.  The  newly  formed  connective  tissue  is  rarely  of  greater  firm- 
ness, and  is  often  less  firm,  than  the  nerve-elements  it  replaces.  In 
sclerosis,  however,  we  must  distinguish  two  types.  In  one  the  process 
is  limited  to  structures  that  have  the  same  function ;  the  process 
begins  as  a  degeneration  of  the  nerve-elements,  and  the  overgrowth 
•of  the  connective  tissue  is  a  consequence  of  their  wasting— the  affection 
is  essentially  "  parenchymatous."  The  so-called  "secondary  degene- 
rations "  are  of  this  character  ;  but  similar  changes  are  often  primary, 
and  affect  the  structures  that  have  the  same  function,  often  through  a 
considerable  extent  of  the  cord.  Hence  they  are  termed  "  system 
diseases."      In  the  other  type  the  morbid  changes  are  not  distributed 


PATHOLOGICAL    DIAGNOSIS.  275 

according  to  function.  Tliey  are  apparently  random  in  incidence,  and 
involve  adjacent  structures  in  consequence  of  contiguity.  These  begin 
in  the  connective  tissue  and  not  in  the  nerve-elements,  which  suffer 
secondarily.  They  are  essentially  interstitial  processes.  Insular 
sclerosis  is  an  example  of  this  type. 

The  position  of  the  islets  of  sclerosis  has  no  relation  to  the  function  of  the 
parts,  and  may  involve  parts  of  adjacent  structures  that  have  no  common  func- 
tion. Areas  of  difEuse  sclerosis  are  also  met  with  which  have  not  the  sharp 
limitation  of  the  insular  form,  and  are  regarded  by  some  as  of  the  nature  of 
chi'onic  sclerotic  inflammation.  If  such  a  lesion  is  so  situated  as  to  involve 
fibres  that  undergo  secondary  degeneration,  this  necessarily  results  when  the 
damage  to  the  fibres  is  sufficiently  great.  We  have  then  a  combination  of  the 
random  and  systemic  forms  of  sclerosis,  but  one  that  has  no  real  pathological 
significance. 

There  are,  however,  other  relations  between  the  two  types  that  deserve 
attention.  These  will  be  considered  in  connection  with  the  special  morbid 
processes  that  present  them,  but  a  brief  mention  of  them  here  may  make  some 
facts  of  other  diseases  more  intelligible.  The  process  of  secondary  degeneration 
and  secondary  sclerosis  varies  in  its  character,  presenting  in  some  oases  a 
greater  amount  of  vascular  disturbance  than  in  othera.  It  seems  to  partake  of 
the  character  of  the  lesion  causing  it,  as  we  have  seen  (p.  73)  the  process 
of  secondary  degeneration  in  the  nerves  may  do.  The  same  difference  is  seen 
in  the  primary  degenerations,  such  as  occur  in  tabes.  This  difference  is 
analogous  to  that  which,  as  already  described,  occurs  in  some  varieties  of 
multiple  neuritis.  We  can  indeed  see  the  difference  in  the  eye :  tabetic 
optic  nerve  atrophy,  in  most  cases,  presents  no  sign  of  inflammation,  while  in 
othei-s  the  early  stage  may  be  accompanied  by  distinct  slight  neuritis,  to  be 
seen  with  the  ophthalmoscope.  Secondly,  the  chronic  inflammations,  with  the 
diffuse  limitation,  extending  to  adjaicent  structures  irrespective  of  function, 
have  yet  sometimes  a  marked  tendency  to  be  localised  in  structures  of  definite 
function.  Thus  there  is  sometimes  diffuse  sclerosis  of  the  lateral  pyramidal 
tracts  and  posterior  median  columns,  not  sharply  limited  to  these,  and  yet  with 
a  correspondence  and  symmetry  not  to  be  overlooked.  Thus  the  two  processes 
tend  to  meet ;  the  systemic  degeneration  or  sclerosis  to  be  diffuse ;  the 
difEuse  inflammation  or  sclerosis  to  be  systemic ;  and  it  is  sometimes  not 
easy  to  say,  from  mere  microscopic  examination,  to  which  class  a  lesion 
belongs.  Lastly,  there  is  a  curious  fact,  which  has  been  strangely  overlooked 
although  attention  was  first  called  to  it  by  Charcot — that  inflammation 
may  extend  from  a  primary  lesion  along  a  tract  that  undergoes  secondary 
degeneration,  but  in  the  opposite  direction  to  that  of  the  degeneration.  It  is 
very  common  for  the  inflammation  at  a  given  place  in  the  cord,  especially  that 
due  to  compression,  to  extend  farthest  along  certain  tracts,  irrespective  of  their 
secondary  degeneration,  and  in  some  cases  this  extension  occurs  through  a  con- 
siderable extent  of  the  cord.  An  ascending  inflammation  limited  to  the  pyra- 
midal tracts  may,  for  instance,  be  traced  through  several  segments.  The  fact 
suggests  that  the  neuroglial  elements  may  share  to  some  extent  a  difference 
that  obtains  between  the  fibres  of  different  tracts,  and  it  may  easily  lead,  and 
probably  has  led,  to  erroneous  conclusions. 

Softening  of  the  spinal  cord  is  very  common.  Whenever  the  nerve- 
fibres  are  broken  up  into  disconnected  globules  of  myelin,  separated 


276  SPINAL   CORD. 

bj  serum  in  a  sort  of  emulsion,  tlie  consistence  of  the  part  is  necea- 
sarily  lessened.  Acute  inflammation  always  causes  such  a  breaking 
up  of  the  nerve-fibres,  and  hence  inflammation  always  causes  softening 
as  its  first  effect.  The  separate  particles  are  augmented  by  leucocytes, 
which  escape  abundantly  into  the  tissue.  Ultimately  connective-tissue 
elements  are  formed,  which  increase  the  consistence,  it  may  be  up  to, 
.  and  even  beyond,  the  normal  degree.  In  a  very  chronic  inflammation 
the  formation  of  new  tissue  may  proceed  pari  passu  with  the  destruc- 
tive process,  and  there  may  be  at  no  time  much  diminution  of 
consistence.  It  is  in  these  cases  that  it  is  difficult  to  draw  the  line 
between  inflammation  and  degeneration. 

Does  such  softening  of  the  cord  occur  apart  from  inflammation  ? 
The  question  is  not  easy  to  answer.  In  the  brain  necrotic  softening 
is  very  common  as  a  consequence  of  arterial  obstruction — is  indeed  the 
common  form  of  softening.  But  in  the  spinal  cord  we  have  no  such 
distinct  evidence  of  the  occurrence  of  necrotic  softening.  If  embolism 
occurs,  it  is  excessively  rare.  The  course  of  the  vessels  does  not 
favour  the  passage  of  a  plug  into  them,  while  the  vertical  connection 
in  the  anterior  spinal  and  anastomotic  arteries  (see  p.  227)  will  prevent 
damage  from  obstruction  in  the  central  system  unless  this  is  situated 
in  the  terminal  vessels  of  the  grey  matter.  Arterial  thrombosis,  due 
to  atheroma  of  the  walls  of  the  vessels,  such  as  is  so  common  in  the 
brain,  probably  does  not  occur  in  the  cord.  The  arteries  are  smaller 
than  those  in  which  atheroma  is  met  with  in  the  brain,  and  the  low- 
ness  of  the  blood-pressure  within  them  involves  the  absence  of  the 
chief  cause  of  atheroma.  It  is  likely  that  spontaneous  thrombosis 
occurring  in  vessels  affected  with  syphilitic  endarteritis  sometimes  sets 
up  the  changes  that  are  now  regarded  as  those  of  primary  inflamma- 
tion; and  such  a  lesion  has  actually  been  met  with,  but  we  have 
no  evidence  of  its  frequency.* 

Besides  the  morbid  processes  to  which  the  cord  itself  is  liable,  it 
suffers  also  in  consequence  of  disease  outside  it.  It  may  be  com- 
pressed by  growths  springing  from  the  membranes  or  bones,  and  by 
products  of  inflammation  within  the  spinal  canal.  Compression  not 
only  causes  degeneration  of  the  nerve-elements,  but  usually  excites 
actual  inflammation.  This  "  compression-myelitis  "  may  attain  a 
degree  and  an  acuteness  out  of  all  proportion  to  the  causal  compres- 
sion. The  membranes  may  be  the  seat  of  haemorrhage  which  com- 
presses the  cord,  or  inflammation  which  irritates  it,  even  when  the 
organ  is  not  invaded  in  appreciable  degree. 

The  series  of  recognised  lesions  of  the  spinal  cord,  obvious  or  micro- 
scopic, does  not  by  any  means  exhaust  the  list  of  its  morbid  states. 
Changes  may  occur  in  the  nutrition  of  its  elements  interfering  with 
their  function,  which  are,  and  are  likely  to  remain,  altogether  beyond 
our  means  of  detection.  Such  morbid  states  are  often  called  "  func- 
tional diseases,"  but  when  prolonged  they  are  probably  more  accurately 
•  Williamson,  •  Lancet,'  1894. 


PATHOLOGICAL  DIAGNOSIS.  277 

-conceived  as  nutritional  diseases  (see  p.  1).  Deiangemeuts  of 
function  that  we  can  regard  as  purely  such  are  few  and  rare.  But 
disturbances  of  function  may  be  due  to  organic  disease  in  some  other 
part  of  the  nervous  system,  and  they  may  also  be  the  result  of  toxic 
iigents. 

Lastly,  functional  action  in  all  organs  is  attended  with  increased 
blood-supply ;  and  when  excessive,  the  vascular  disturbance  may  go  on  to 
a  condition  indistinguishable  from  inflammation,  with  escape  of  leuco- 
cytes, &c.  The  prolonged  experimental  stimulation  of  sensory  nerves 
has  actually  produced  myelitis  in  the  related  portion  of  the  cord. 

In  connection  with  the  general  pathology  of  diseases  of  the  spinal 
cord,  it  is  important  to  note  certain  general  facts  of  their  causation : — 
(1)  Neuropathic  disposition. — An  inherited  tendency  to  disease  of  the 
nerve-elements,  manifested  by  such  affections  as  epilepsy  and  insanity. 
This  cause  is  chiefly  influential  in  producing  the  structural  and 
nutritional  diseases,  beginning  in  the  nerve-elements,  and  especially, 
among  structural  diseases,  the  "  system-degenerations,"  as  they  are 
called.  (2)  Prolonged  mental  distress  sometimes  sets  up  degenerative 
changes  in  some  elements  of  the  cord,  especially  in  persons  who  are 
predisposed  by  (1).  (3)  Injury. — A  severe  concussion  of  the  cord 
may  cause  (a)  instant  grave  damage,  usually  haemorrhage.  Or  (b)  it 
may  cause  no  immediate  effect,  but  symptoms  may  come  on  at  the 
end  of  a  few  days  and  progress  slowly  or  rapidly.  Such  symptoms 
are  usually  due  to  inflammation,  secondary  to  minute  spots  of  injury, 
too  small  to  cause  direct  symptoms.  Lastly,  (c)  the  concussion 
sometimes  seems  to  pervert  the  process  of  nutrition  in  the  nerve- 
elements.  Slow  symptoms  of  impaired  function  may  result,  and 
these  may  progress  until,  after  months  or  years,  there  is  actual 
structural  disease.  Similar  effects,  especially  the  graver  varieties,  are 
met  with  as  a  result  of  lessened  atmospheric  pressure  in  divers  and 
workers  in  caissons.  (4)  Exposure  to  cold. — Wet  cold  is  especially 
effective.  The  most  frequent  effect  of  a  severe  exposure  is  acute 
inflammation,  but  habitual  exposure  may  produce  chronic  inflamma- 
tion, or  degenerative  disease.  The  exposure  that  is  effective  may  be 
general,  or  chiefly  of  the  feet  and  legs,  occasionally  of  the  back.  (5) 
The  spinal  cord  appears  also  to  be  the  seat  of  primary  infection  as 
in  epidemic  cerebro-spinal  meningitis,  tetanus,  and  possibly  acute 
anterior  polio-myelitis.  (6)  Toxic  agents  that  can  act  on  the  cord  are 
numerous,  and  are  seen  in  alcoholism  and  lathyrism.  (7)  Other 
diseases  may  also  induce  morbid  changes  in  the  spinal  cord.  The 
primary  malady  may  be  an  acute  specific  disease,  or  some  more 
obscure  blood-state,  the  precise  nature  and  relations  of  which  are  still 
undecided.  It  is  probable  that  certain  blood-states  due  to  an  organ- 
ised virus  excite  some  forms  of  inflammation  of  the  cord  and  of  its 
membranes,  just  as  it  may  cause  inflammation  of  the  peripheral  nerves. 
Septicaemia  may  excite  meningitis,  or  even  abscess  of  the  cord  itself. 
Tubercle  is  common  on  the  m.embranes,  but  seldom  produces  iniiam- 


278  SFINAL    CORD. 

mation.  Diphtheria  may  cause  acute  clianges  in  the  nerve-cells  and 
nerve-roots.  Gonorrhoea  *  has  produced  myelitis,  and  such  diseases 
as  variola,  typhus,  &c.,  have  been  followed  by  cord  inflammation. 
Syphilis  is  a  very  frequent  cause  of  disease,  and  may  act  in  several 
ways.  The  demonstrated  mechanisms  are  (a)  by  a  syphilitic  growth 
compressing  or  invading  the  cord,  (6)  by  changes  in  the  cord 
resulting  from  thrombosis  occurring  in  diseased  vessels,  and  (c)  by 
chronic  syphihtic  meningitis,  damaging  the  cord  and  the  nerve-roots. 
But  (d)  acute  and  chronic  inflammations  of  the  cord  are  often  met 
with  in  syphilitic  subjects,  and  have  been  thought  to  be,  in  many 
instances,  of  syphilitic  origin.  It  is  doubtftd  whether  they  present 
any  syphilitic  characters,  i.  e.  any  histological  features  by  which  they 
differ  from  non- syphilitic  inflammations.  The  evidence  of  dependence 
on  syphilis  is  stronger  in  the  case  of  chronic  and  subacute  disseminated 
inflammation  than  in  the  case  of  acute  myelitis.  It  may  be,  however, 
that  inflammations  due  to  other  causes,  e.  g.  exposure,  run  a  more  acute 
course  when  they  affect  syphilitic  subjects.  The  part  thus  played  by 
syphilis  in  lessening  the  resisting  j)ower  of  the  organism  may  be 
shared  by  other  factors,  such  as  malnutrition  and  excesses  of  various 
kinds.  Lastly,  (e)  certain  degenerative  diseases  of  the  cord  are  very 
commonly  preceded  by  syphilis  ;  one  of  them,  locomotor  ataxy,  so 
frequently  that  a  causal  relation  between  the  two  can  scarcely  be 
doubted.  But  these  degenerative  diseases  are  certainly  not  syphilitic 
in  nature ;  they  differ  in  no  respect  from  the  similar  morbid  processes 
that  occur  in  individuals  who  have  not  had  syphilis,  and  are  probably  the 
consequence  of  some  toxin  left  behind  by  the  morbid  agency  to  which 
the  constitutional  malady  is  due.  (8)  Excessive  muscular  exertion 
has  been  held  to  play  a  part  in  the  generation  of  chronic  disease  of  the 
cord.  This  view  has  of  late  derived  some  support  from  experiments 
iipon  animals. t  (9)  Pathological  changes  in  the  cord  are  known  to 
result  from  the  amputation  of  limbs.  These  have,  however,  no 
clinical  significance. 

The  outline  just  given  of  the  general  pathology  and  etiology  of 
diseases  of  the  spinal  cord  wiU  enable  us  to  consider  the  last  element 
in  diagnosis,  the  nature  of  the  lesion.  The  seat  of  the  disease  is 
indicated  by  the  combination  of  symptoms;  its  nature  can  only  be 
determined  by  considering,  separately  and  together,  several  other 
points : — (1)  The  way  in  which  the  symptoms  came  on.  (2)  The 
causes  that  can  be  traced,  taken  in  conjimction  with  the  known  effects 
of  those  causes.  (3)  The  seat  of  the  disease,  taken  in  conjunction 
with  the  known  liability  of  certain  structures  to  certain  lesions.  The 
most  important  of  these  elements  is  the  mode  of  onset,  and  the  other 
indications  should  only  be  used  in  strict  subordination  to  this. 

*  Gowers,  '  Clinical  Lectures  on  Neivous  Diseases,'  p.  119 ;  also  Leyden  and 
Goldscheider,  '  Die  Erkrankungen  des  Riickeniuarkes,'  p.  369. 

t  Independently  by  O.  Rosenbach  and  Edinger  (see  Edinger  and  Helbing,. 
'  Verhandlungen  des  16  Cong,  f .  inner.  Med.,'  Wiesbaden,  1898). 


PATHOLOGICAL  DIAGNOSIS.  279 

The  time  occupied  by  the  onset  of  the  disease  is  thus  the  first 
element  in  the  pathological  diagnosis.  By  "  time  of  onset "  is  meant 
the  period  that  elapses  between  the  actual  commencement  of  the 
symptoms  and  their  attainment  of  a  considerable  degree  of  intensity. 
We  may  divide  the  chief  modes  of  onset  into  five  classes,  and 
classify  the  most  common  lesions  in  relation  to  them,  in  the  following 
table : 

DiSBASB.  Onset.  Disease. 

Sudden  T 

(few  minutes)  [  ^^        i      i    • 

J     .  '  avascular  I0S10U8. 

Acute  I 

(few  hours  or  days)        J  ~) 

r  Subacute  | 

I      (one  to  six  weeks)  i» Inflammation, 

Pressure  and  J  Suhchronic  J 

growths      }       (six  weeks  to  six  months)  J  "1 

Chronic  i- Degeneration, 

[_     (more  than  six  months)         J 


A  lesion  of  sudden  occurrence,  the  symptoms  developing  in  the 
course  of  a  few  minutes,  is  almost  always  vascular,  commonly  haemor- 
rhage, sometimes  perhaps  vascular  obstruction.  But  a  vascular  lesion 
may  occupy  a  somewhat  longer  time  in  development — a  few  hours  or 
days.  In  acute  and  subacute  inflammation  the  symptoms  come  on  in 
the  course  of  a  few  hours,  a  few  days,  or  a  few  weeks.  Subacute  and 
chronic  inflammation  occupies  from  a  few  weeks  to  a  few  months. 
Degeneration,  in  which  there  is  no  adequate  evidence  of  any  inflam- 
matory process,  occupies  many  months,  or  it  may  be  years.  The  sym- 
ptoms produced  by  tumours  which  invade  or  compress,  and  by  simple 
pressure  (traumatic  causes  excluded),  are  never  sudden  or  very  acute, 
and  rarely  very  chronic ;  the  time  occupied  by  the  development  of  tho 
symptoms  varying,  according  to  the  nature  of  the  cause,  from  a  fort- 
night to  six  months. 

It  is  necessary  to  consider,  however,  not  merely  the  whole  time 
occupied  by  the  development  of  the  disease,  but  also  the  uniformity  of 
its  course.  Two  or  more  morbid  processes  may  concur.  An  initial 
myelitis,  for  instance,  may  lead  to  a  secondary  degeneration ;  and  on 
the  other  hand,  in  degenerated  tissues,  sudden  vascular  lesions  oc- 
casionally occur.  Pressure  produces  local  myelitis,  which  may  be 
independent  of  the  pressure  in  its  development,  and  have  an  acute  or 
subacute  onset.  The  whole  course  of  the  disease  must  be  ascertained 
before  an  inference  is  drawn,  and  the  possibility  of  a  double  process 
must  always  be  kept  in  view. 

The  onset  and  course  of  the  symptoms  thus  sometimes  enable  us  to 
decide  at  once  that  a  lesion  is  of  a  given  character,  as  that  one  which 
occurs  instantly  is  vascular,  or  that  one  which  takes  years  for  its 
development  is  degenerative.  More  frequently  they  enable  us  to 
exclude  certain  morbid  processes,  and  to  restrict  the  possible  lesion  to 
two  or  three  forms.     For  instance,  a  lesion  which  comes  on  in  the 


280  SPINAL   CORD. 

course  of  a  few  hours  must  be  either  vascular  or  inflammatoryc 
Between  these  we  have  to  decide  by  attention  to  other  indications. 

In  actual  diagnosis  it  is  convenient  to  consider  next  the  indication 
afforded  by  the  position  and  distribution  of  the  disease.  We  consider 
what  diseases  occur  in  this  situation,  and  then  which  of  them  have  the 
mode  of  onset  that  has  been  ascertained.  Th^s  indication  involves  a 
knowledge  of  the  various  diseases  and  their  seat  The  most  important 
consideration  is  that  a  wide  range  of  symptoms  jf  uniform  character 
indicates  the  affection  of  a  definite  system  of  structure,  and  in  most 
instances  a  disease  commencing  in  the  nerve-elements,  and  if  the 
onset  be  chronic  we  may  feel  sure  that  it  is  a  degeneration.  On  the 
other  hand,  the  involvement  of  many  functions  suggests  a  random 
process,  such  as  inflammation  or  pressure.  But  this  indication  is 
always  to  be  subordinated  to  the  mode  of  onset.  Thus  the  limitation 
to  a  single  structure  does  not  exclude  inflammation :  this  may  affect, 
for  instance,  the  anterior  grey  matter  only,  and  cause  corresponding 
symptoms. 

The  symptoms  may  indicate  a  morbid  process  limited  to  one  half  of 
the  cord,  but  this  does  not  materially  modify  the  diagnostic  method. 
Almost  any  process  may,  in  rare  cases,  be  thus  limited.  System 
degenerations  and  acute  inflammations  are  least  frequently  unilateral, 
and  they  never  reach  a  considerable  degree  of  intensity  on  one  side 
without  some  affection  of  the  other  side  On  the  other  hand,  tumours 
and  foci  of  chronic  myelitis  are  often  one-sided,  and  still  more  often 
affect  one  half  of  the  cord  first  and  then  the  other. 

Indication  of  disease  outside  the  cord,  irritation  of  certain  nerve- 
roots,  causing  severe  local  pain,  often  precedes  the  symptoms  of  com- 
pression, and  is  an  important  aid  to  diagnosis.  It  shows  the  existence 
of  a  morbid  process  outside  the  cord  before  the  cord  is  involved.  But 
we  cannot  use  even  this  indication  except  in  dependence  on  the  mode 
of  onset.  A  disease,  as  a  growth  outside  the  cord,  may,  as  we  have 
seen,  not  only  compress  the  cord,  and  cause  slow  loss  of  power ;  it 
may  excite  inflammation  and  cause  rapid  palsy. 

The  last  element  in  the  pathological  diagnosis  is  the  detection  of 
any  influence  which  can  be  regarded  as  the  cause  of  the  disease  in  the 
spinal  cord,  or  any  associated  condition  which  may  indicate  an  active 
morbid  process.  We  have  seen  that  the  mode  of  onset  may  help  us 
to  limit  the  disease  to  certain  possible  forms  of  lesion ;  the  distribu- 
tion of  the  affection  may  render  it  probable  that  it  is  one  or  other  of 
these  forms ;  and  the  detection  of  a  cause  and  the  knowledge  of  the 
lesion  which  that  cause  produces  may  help  us  to  carry  the  diagnosis  still 
further.  The  most  important  general  causes  of  disease  of  the  cord, 
and  the  processes  to  which  they  chiefly  give  rise,  have  been  already 
mentioned.  The  causal  element  in  diagnosis  is  chiefly  an  application 
of  those  facts. 

The  morbid  process  outside  the  spinal  cord  that  most  closely  simu- 
lates its  disease  is,  unquestionably,  parenchymatous  multiple  neuritis. 


INJURIES    OF   THE    SPINE.  281 

The  diagnosis  depends  on  a  tliorougli  knowledge  of  the  varied  sym- 
ptoms of  the  latter,  since  its  elements  vary  according  to  the  different 
manifestations  of  the  disease  of  the  nerves.  For  these,  and  for  any 
general  diagnostic  principles,  the  reader  is  referred  to  the  account  of 
that  disease. 

The  only  sure  ground  for  diagnosis  is  a  thorough  knowledge  of  the 
various  morbid  processes  and  their  symptoms ;  and  the  only  safe  plan 
is  to  work  by  these,  from  symptom  to  seat  and  onset  to  nature, 
treating  every  case  as  a  problem  to  be  worked  out  to  a  definite 
diagnosis,  and  only  then  comparing  the  result  with  the  types  of 
disease.  To  one  of  these  the  case  may  or  may  not  conform  ;  if  it  does 
not,  the  comparison  with  types  as  a  means  of  diagnosis  will  only  leave 
the  observer  stranded  and  powerless. 

The  distinction  of  functional  and  nutritional  disease  from  organic 
lesions  may  conveniently  be  postponed  until  the  symptoms  of  the 
former  are  specially  described. 


SPECIAL  DISEASES  OF  THE  SPINAL  CORD. 

DISEASES  OF  THE  VEBTEBBAL  COLUMN. 

Diseases  of  the  bones  of  the  spine  fall  for  the  most  part  within  the 
province  of  surgery.  But  there  are  few  of  these  diseases  that  do  not, 
among  their  most  frequent  effects,  interfere  with  the  functions  of  the 
spinal  cord.  Hence  an  account  of  the  diseases  of  the  cord  would  be 
incomplete  without  some  mention  of  the  morbid  states  that  begin  in 
its  bony  case. 


INJURIES  OF  THE  SPINE. 

Injuries  to  the  spinal  column  may  consist  of  punctured  wounds 
(which  need  not  be  considered  here),  concussion  (the  effects  of  which 
on  the  cord  will  be  considered  later),  and  fracture  or  dislocation  of 
the  vertebrae.  It  may,  however,  be  useful  briefly  to  describe  the  more 
salient  features  of  the  last  two  conditions,  their  relation  to  the  damage 
the  cord  sustains,  and  the  symptoms  thus  produced. 

Dislocation. 

Simple  dislocation  occurs  in  the  cervical  region,  most  frequently  at 
the  fifth  and  sixth  vertebrae.  It  may  take  place  gradually  or  suddenly: 
gradual  displacement  is  always  secondary  to  disease  of  the  bones; 


282  SPINAL    COliD. 

mdden  displacement  may  occur  in  disease  or  from  injury.  The 
damage  to  the  cord  is  always  greatest  in  traumatic  displacement  of 
healthy  bones,  because  the  force  needed  to  produce  the  dislocation  ia 
much  greater,  the  displacement  is  more  considerable,  and  the  effect 
on  the  canal  and  the  contained  cord  is  greater.  Displacement  may 
occur  in  any  direction :  it  usually  iuYolves  both  vertebral  articulations, 
but,  in  rare  cases,  it  is  oblique,  involving  one  articulation  only.  The 
common  causes  are  violent  blows  or  falls  on  the  head,  rarely  sudden 
rotation  of  the  head  while  a  weight  is  carried  upon  it.  The  symptoms 
are  a  lateral  or  forward  or  backward  displacement  of  the  head,  so 
that  the  chin  is  in  contact  with  the  shoulder  or  the  chest,  or  the 
occiput  with  the  nape  of  the  neck.  There  is  also  irregularity  of  the 
vertebral  spines,  usually  readily  detected.  The  cord  is  damaged  in 
most  cases,  and  the  symptoms  are  those  of  a  total  transverse  lesion 
in  the  situation  of  the  luxation.  It  may  be  merely  compressed, 
especially  in  cases  of  disease,  in  which  the  displacement  has  occurred 
with  little  force.  The  symptoms  of  paralysis  have  been  known  to 
pass  away,  in  such  a  case,  on  the  reduction  of  the  dislocation.  More 
commonly  the  cord  is  also  bruised,  with  extravasation  of  blood,  and 
secondary  myelitis  occurs  later.  In  such  cases,  if  the  patients  live, 
there  may  be  anaesthesia  or  hyperaesthesia  below  the  lesion,  with  total 
paralysis  of  the  limbs,  and  excess  of  reflex  action.  In  rare  instances 
the  cord  has  been  completely  divided.  In  still  rarer  instances  of  slight 
displacement  it  has  not  been  injured. 

Rupture  of  the  transverse  ligament  which  retains  the  odontoid 
process  may  permit  the  latter  to  compress  the  cord,  and  thus  to  cause 
instant  death.  This  often  results  from  sudden  suspension  by  the 
head,  as  in  crimir.%xl  executions.  One  of  the  curiosities  of  surgical 
literature  is  a  case  related  by  Petit  in  which  a  man,  playing  with  a 
neighbour's  child,  lifted  it  up  by  the  head,  and  caused  instant  death 
by  rupturing  the  transverse  ligament.  The  father  of  the  child, 
entering  at  the  moment,  stabbed  the  man  with  a  knife,  the  blade 
of  which  passed  in  between  the  first  and  second  cervical  vertebrae, 
divided  the  spinal  cord,  and  the  man  also  fell  dead.  Rupture  of  the 
ligament  has  also  resulted  from  raisiag  a  heavy  weight  with  the  head. 
The  treatment  of  dislocation  is  too  purely  surgical  to  be  described 
here. 

Fractttre. 

All  organic  diseases  of  the  bones,  weakening  them,  predispose  to 
fracture.  Among  these,  one  is  of  special  medical  interest ;  the  ver- 
tebrae, especially  in  the  lumbar  region,  may  share  the  rarefaction  and 
weakening  of  the  osseous  tissues  occasionally  produced  in  tabes,  and 
then  a  very  slight  traumatic  influence,  a  blow  or  wrench,  may  cause 
fracture.  The  force  needed  is  often  so  slight  as  to  fail  to  cause  dis- 
placement or  other  symptoms  except  local  pain,  and  symptoms  may 
only  supervene  at  a  somewhat  later  date.     Apart  from  disease,  the 


INJUEIES    OP    THE    SPINE.  28S 

accident  is  most  common  in  adults,  tlie  greater  elasticity  of  the  verte- 
bral column  in  children  giving  to  them  a  comparative  immunity.  Its 
cause  is  a  blow  or  fall  on  the  spLae,  or  sudden  forcible  flexion.  In 
extremely  rare  cases  a  fracture,  "dually  slight,  has  resulted  from 
severe  muscular  exertion.  This  i  an  importanb  fact,  showing  that 
muscular  exertion  may  injure  the  spine,  and  may  lead  to  symptoms 
at  a  later  date.  Symptoms  of  such  injury,  in  slight  degree,  are  not 
uncommon. 

Fracture  may  occur  at  any  part  of  the  spine,  but  is  most  frequent 
at  the  fifth  or  sixth  cervical,  and  at  the  last  dorsal  or  first  lumbar 
vertebrse.  In  the  dorsal  and  lumbar  regions  the  bodies  are  broken 
in  two  thirds  of  the  cases,  but  in  the  cervical  region  the  arches  alone 
are  fractured  in  one  half.  Usually  there  is  a  displacement  of  the 
vertebral  column  at  the  seat  of  fracture.  Earely  the  bodies  may  be 
crushed  without  displacement.  The  displacement  of  the  bone  involves 
a  narrowing  of  the  canal  and,  usually,  compression  of  the  cord.  This 
may  also  result  when  the  arches  only  are  driven  in.  But  the  cord 
may  be  seriously  damaged  when  there  is  no  permanent  narrowing  of 
the  canal,  as  in  Figs.  89  and  90.    The  dura  mater  is  rarely  torn  except 


<^ 


Pig.  89. — Frac  hi  re  of  the  first  lumbar  vertebra.  DM.  Dura  mater.  There 
was  no  perniiuit^nt  narrowing  of  the  canal,  but,  nevertheless,  the  spinal 
cord  was  greatly  damaged  at  the  spot;  see  next  figure. 

bj  a  splinter.  Blood  is  almost  always  extravasated  outside  the  dura 
mater,  often  in  considerable  quantity,  from  the  rupture  of  the  large 
veins  in  this  situation.  There  are  usually  only  small  extravasations 
in  the  pia  mater.  The  cord  is,  in  most  cases,  bruised  and  compressed 
by  the  lower  fragment  (Fig.  91).  Sometimes  it  is  flattened,  and  it 
may  even  be  divided,  all  nerve- substance  being  squeezed  out  of  the 
pia-matral  sheath  at  the  spot.  In  the  case  shown  in  Figs.  89  and  90 
the  cord  appeared  to  have  been  split  longitudinally  at  the  spot,  perhaps 
by  the  mere  force  of  the  concussion.  It  is  very  common  to  have  local 
myelitis,  opposite  the  fracture,  without  any  permanent  narrowing  of 
the  canal  or  compression  of  the  cord  j  the  inflammation  seems  to  be 


284  SPINAL    CORD. 

tlie  direct  result  of  tlie  concussion.  Blood  may  be  extravasated  into 
tlie  bruised  part,  sometimes  in  minute  spots,  sometimes  in  larger 
haemorrliages,  and  even  into  tbe  central  canal.  These  changes  are 
usually  limited  to  the  spot  directly  damaged,  but  secondary  myelitis 
may  be  set  up,  and  sometimes  extends  beyond  the  contused  area.  It 
Ft  a.  90.  Fig.  91. 


I 


Fia.  90. — Spinal  cord  damaged  by  the  fracture  shown  in  Pig.  89.  The  elements 
of  the  cord  itself  are  cbano^ed  beyond  the  possibility  of  identification.  The  ascending 
degeneration  is  shown  in  Fig.  76. 

Pig-.  91. — Fracture  of  tlie  body  of  the  fifth  dorsal  vertebra  and  of  its  processes. 
(After  Gurlt.) 

may  even  extend  through  the  whole  length  of  the  cord  below  the 
injury,  and  then  its  central  functions  are  abolished.  In  cases  of 
some  duration  the  usual  ascending  and  descending  secondary  dege- 
nerations are  also  found.  It  is  important  to  note,  moreover,  that 
the  secondary  degenerations  have  sometimes  the  irritative  character 
already  described  (p.  272,  note),  and  that  an  ascending  inflammation 
may  for  a  short  distance  above  the  lesion  be  limited  to  a  tract  that 
degenerates  downwards  (see  p.  275).  Fig.  *1^,  p.  214,  represents 
sections  of  the  cord  from  the  case  of  fracture  figured  above.  Occa- 
sionally the  injury  leads  to  secondary  caries  of  the  bone,  with  all  its 
consequences. 

Symptoms. — Three  classes  of  symptoms  result.  (1)  The  local  indica- 
tions of  the  injury  to  the  spine.  (2)  There  may  be  certain  nervous 
symptoms  not  distinctly  due  to  the  damage  to  the  cord.  One  of  these 
is  general  shock,  which  may  be  so  great  as  to  entail  transient  loss  of 
consciousness.  Vomiting  occasionally  occurs.  There  is  great  pain  in 
the  position  of  the  fracture,  rendered  very  intense  by  pressure,  and 
often  radiating  along  the  nerves  which  come  from  this  part,  the 
roots  of  which  are  compressed.  In  rare  cases  epileptiform  con- 
vulsions have  followed  fracture,  usually  at  an  interval  of  some  days. 
(3)  Symptoms  which  result  from  the  damage  to  the  cord,  and  con- 
sist in  paralysis  of  the  parts  below  the  injury.     Its  character  depends 


INJUEIES    OF   THE    SPINE.  285 

on  the  amount  of  damage.  If'  this  is  considerable,  there  is  both 
motor  and  sensory  paralysis  up  to  the  level  of  the  lesion,  with  loss  of 
power  over  the  sphincters.  Reflex  action  is  lost  at  the  level  of  the 
lesion,  and  the  examination  of  the  trunk- reflexes  often  gives  important 
information  regarding  the  extent  of  the  damage,  when  this  is  in  the 
dorsal  region.  Below,  reflex  action  is  usually  diminished  or  lost, 
probably  because  the  centres  are  impaired  by  descending  myelitis. 
Spasmodic  twitchings  sometimes  occur  in  the  limbs  immediately  after 
the  injury,  and  priapism  is  the  rule,  especially  if  the  lesion  is  cervical. 
The  pains  are  severe  in  the  arms  when  the  fracture  is  opposite  the 
cervical  enlargement,  and  in  the  legs  when  at  or  below  the  lumbar 
enlargement,  so  as  to  damage  the  nerve-roots.  In  these  cases  there 
may  be  rapid  wasting  of  the  muscles,  with  loss  of  electric  irritability. 
There  is  usually  at  first  incontinence,  afterwards  retention  of  urine, 
but  the  former  is  persistent  if  the  lumbar  centres  are  damaged. 
Cystitis,  bedsores,  &c.,  may  supervene.  ITltimately,  if  the  damage  is 
above  the  lumbar  enlargement  and  the  patient  lives,  there  may  be 
increased  myotatic  irritability  in  the  limbs,  progressing  to  spasm,  so 
that  spastic  paraplegia  results.  In  many  cases  in  which  the  knee-jerk 
is  at  first  lost  it  may  be  months,  or  even  years,  before  it  returns. 

Special  symptoms  result  when  the  injury  is  in  certain  parts  of  the 
spine.  Fracture  of  the  first  two  cervical  vertebrae  causes  instant  death, , 
unless  the  displacement  is  very  slight,  and  even  then  there  is  imminent 
danger  of  further  displacement,  with  the  most  serious  consequences, 
on  any  incautious  voluntary  movement.  With  slight  displacement 
persons  have  been  known  to  live  for  weeks,  and  then  die  from  secon- 
dary myelitis ;  they  have  even  recovered  altogether.  Now  and  then 
there  is  no  compression  of  the  cord,  although  there  is  distinct  displace- 
ment, even  sufficient  to  be  recognised  in  the  pharynx  (Ley den).  In 
such  a  case  death  has  resulted  at  a  later  period  from  caries.  The 
characteristic  symptoms  are  local  pain,  increased  by  all  movements 
(which  are  rendered  almost  impossible),  displacement,  and  spinal 
symptoms.  The  latter  may  be  slight — merely  difficulty  in  breathing 
or  swallowing — or  considerable,  and  involving  the  trunk  and  limbs. 
Sometimes  there  is  hyperpyrexia.  Not  more  than  one  case  in  fifty 
recovers. 

Middle  Cervical  Vertehrse. — The  third,  fourth,  and  fifth  vertebrae  are 
most  frequently  fractured.  When  the  injury  to  the  cord  is  consider- 
able, death  usually  occurs  very  rapidly,  because  the  roots  of  the  phrenic 
nerve  are  involved,  the  intercostals  being  necessarily  paralysed  with 
the  parts  below.  In  some  cases  there  is  little  immediate  displacement, 
and  the  symptoms  are  slight  until  further  displacement  occurs  in 
some  movement.  Thus  a  man  who  had  met  with  an  injury  of  this 
kind  went  to  be  shaved ;  during  the  proceeding,  his  head  was  turned 
on  one  side  by  the  barber,  with  the  unexpected  restilt  of  causing  dis- 
placement of  the  fracture,  and  immediate  death.  When  the  fracture 
is  at  the  cervico-dorsal  region,  opposite  the  lower  part  of  the  cervical 


286  DISEASES   OF  THE   SPINE. 

enlargement,  tlie  arms  frequently  escape  at  first,  tlie  early  paralysis 
being  confined  to  the  legs  and  muscles  of  the  trunk.  Respiration  is 
diaphragmatic  only.  After  a  few  days  the  arms  become  involved,  but 
theii-  paralysis  is  often  partial,  affecting,  for  instance,  only  certain 
muscles,  as  the  extensors  of  the  hand,  and  it  is  often  accompanied  by 
local  spasmodic  movements.  Movements  and  pressure  cause  pain, 
and  there  is  local  muscular  rigidity.  The  head  may  be  in  normal  or 
in  abnormal  position.  Yaso-motor  disturbance  in  the  face  and  general 
hyperpyrexia  have  been  observed.  There  is  not  unfrequently  altera- 
tion in  the  joints,  especially  the  knee-joints,  apparently  the  result  of 
the  presence  of  fluid  in  them. 

In  fracture  of  the  dorsal  vertebrae  (2 — 11)  the  arms  escape,  the  legd 
are  paralysed,  and  the  trunk-muscles  up  to  the  height  of  the  lesion. 
The  pain  in  the  trunk  may  be  very  severe.  There  is  hyperesthesia  or 
anaesthesia  in  the  parts  below.  The  reflex  action  in  the  legs  is  exces- 
sive ;  that  in  the  trunk  is  abolished  at  the  level  of  the  lesion.  The 
last  dorsal  and  first  lumbar  vertebrse  are  fractured  more  frequently 
than  the  others,  and  there  result  paralysis  of  the  legs,  complete  or 
irregular,  severe  pains,  tingling,  &c.,  sometimes  followed  by  hyper- 
sesthesia  or  loss  of  sensibility,  and  by  rapid  disturbance  of  nutrition, 
in  both  the  muscles  and  the  skin.  In  fracture  of  the  lower  lumbar 
vertebrse  the  symptoms  are  often  slight ;  below  the  extremity  of  the 
cord  the  nerves  occupy  a  smaller  space  in  the  canal,  and  so  may  escape 
compression  by  a  moderate  displacement.  If  they  suffer,  the  fractured 
vertebrse  unite  very  slowly.  A  false  joint  is  occasionally  formed. 
Secondary  myelitis  and  its  consequences  are  frequent  causes  of  death 
at  a  variable  period  after  the  injury. 


CAEIES   OF  THE   SPINE. 


Caries  of  the  bones  of  the  spine  is  a  frequent  cause  of  paraplegia. 
It  is  often  termed  "Pott's  disease,"  from  the  English  surgeon,  Percival 
Pott,  who  first  described  it  (in  1779)  as  a  cause  of  paralysis. 

Causes. — Males  are  said  to  be  rather  more  liable  than  females,  but 
the  difference  in  sexual  incidence  is  not  great.  It  is  more  common  in 
childhood  (after  three),  and  next  in  early  adult  life,  but  it  may  occur 
at  any  age,  and  is  perhaps  more  common  in  the  second  half  of  life  than 
any  other  scrofulous  lesion.  I  have  known  it  commence  at  fifty,  and 
it  has  been  met  with  as  late  as  seventy.  It  is  distinctly  a  manifesta- 
tion of  the  tubercular  and  scrofulous  diathesis,  and  evidence  of  such 
inheritance  is  to  be  traced  in  most  cases.  Occasionally  the  sufferer 
himself  presents  such  indications,  e.  g.  lung  disease,  or  caries  of  other 
bones.  It  occasionally  develops  simultaneously  with  other  signs  of 
acute  general  tuberculosis.  Injuries  seem  frequently  to  excite  the 
bone  mischief  in  the  spine,  as  they  certainly  do  analogous  bone  disease 


CARIES.  287 

elsewtere,  in  those  wlio  are  predisposed;  possibly,  sometimes,  in 
healthy  persons.  Falls,  blows  on  the  back,  and  severe  strains  are  the 
most  frequent  traumatic  antecedents.  The  last  may  act  by  straining 
the  ligaments  and  setting  up  inflammation,  which  spreads  to  the  bones 
directly  or  through  the  intervertebral  cartilages.  There  is  usually 
an  interval,  sometimes  of  many  months,  between  the  injury  and  the 
definite  symptoms  of  bone  disease. 

Other  causes  besides  scrofulous  disease  have  occasionally  been 
known  to  cause  breaking  down  of  the  bodies  of  the  vertebrse  with 
angular  curvature  and  all  its  consequences.  Soft  growths  in  the 
spine  and,  in  rare  cases,  syphilitic  formations  have  had  the  same 
effect.  Osteitis,  due  to  extension  from  adjacent  inflammation,  is  a 
rare  cause ;  thus  caries  of  the  cervical  spine  has  been  secondary  to  a 
primary  retro-pharyngeal  abscess.  The  disease  sometimes  follows 
septicaemia,  probably  in  consequence  of  a  secondary  septicsemic 
abscess  in  the  bone.  This  was  the  case  in  a  surgeon  about  sixty 
years  of  age,  in  whom  symptoms  of  blood-poisoning  followed  an  injury 
to  the  ankle.  They  were  succeeded  by  paraplegia  of  rapid  onset, 
and  this  by  the  development  of  angular  curvature  in  the  lower  dorsal 
region.     In  a  large  number  of  cases  no  exciting  cause  can  be  traced. 

Pathological  Anatomt. — The  changes  in  the  bones  need  not 
detain  us,  since  they  belong  to  surgical  pathology,  except  in  so  far  as 
they  lead  to  damage  to  the  cord.  There  is  inflammation  of  the  bodies 
of  the  vertebrae,  and  of  the  intervertebral  substance,  often  at  first 
with  enlargement,  afterwards  with  breaking  down  of  the  substance  of 
the  bone,  which  gives  way  under  the  pressure  to  which  it  is  exposed, 
so  that  deformity  of  the  spine  results.  Products  of  inflammation  and 
destruction  of  tissue,  more  or  less  purulent  in  character,  accumulate 
either  inside  the  canal  or  outside  the  vertebral  column.  In  the  latter 
situation  they  are  increased  in  quantity  by  the  inflammation  they 
excite,  and  the  well-known  forms  of  abscess  result. 

Within  the  canal  the  inflammatory  products  have  a  firmer  con- 
sistence. Although  the  inflammation  extends  to  the  loose  cellular  and 
adipose  tissue  between  the  bone  and  the  dura  mater,  and  often  to  the 
dura  mater  itself,  it  has  little  tendency  to  spread  as  a  purulent  menin- 
gitis, or  even  to  penetrate  the  dura  mater,  the  outer  layer  of  which 
becomes  irregularly  thickened  in  the  neighbourhood  of  the  disease 
(see  Fig.  93),  while  the  inner  surface  of  the  membrane  remains  normal. 
The  firm  consistence  of  the  inflammatory  products,  often  caseous  in 
character,  is  an  important  pathological  feature,  because  this  material 
is  so  often  the  agent  by  which  the  cord  is  compressed. 

The  displacement  that  results  from  the  disease  varies  according  to 
the  extent  and  character  of  the, disease.  Occasionally  there  is  simply 
a  lateral  displacement — one  spine  is  a  little  to  one  side  of  that  above 
it.  More  commonly  the  collapse  of  the  bodies  leads  to  "angular 
curvature,"  the  spinal  column  is  bent  forwards  at  an  acute  angle, 
and  one  or  two  vertebral  spines  are  much  more  prominent  than  the 


288 


DISEASES  OF  THE    SPINE. 


others.  There  is  often,  however,  a  less  abrupt  bend;  the  curvature 
may  extend  over  four  or  five  vertebrse.  Less  commonly  one  spine 
projects  more  than  the  others  without  any  curvature. 

The  nerves,  as  they  pass  through  the  membranes  and  intervertebral 
foramina,  are  irritated  by  the  inflammation,  and  often  compressed  by 
the  thickening  of  the  dura  mater  which  sheaths  them,  and  those 
passing  by  the  seat  of  compression  may  also  suffer  from  the  narrow- 
ing of  the  canal.  They  may  be  foimd  red  and  swollen,  or  shrunken 
and  grey.  They  may  be  damaged  when  the  cord  is  normal,  or  but 
little  affected  when  this  is  compressed. 

The  damage  to  the  spinal  cord  depends  on  the  secondary  conse- 
quences of  the  caries,  and  is  variable  and  uncertain  in  both  occurrence 
and  character.  The  mechanism  of  the  damage  is  twofold — compression 
and  inflammation.  The  relation  between  the  two  is  considered  more 
fuUy  in  a  subsequent  chapter  (Compression  of  the  Spinal  Cord). 
Slow  compression  may  be  attended  by  chronic  inflammation,  secon- 
dary not  only  in  character  but  in  course,  or  by  an  acute  inflammation 

out  of  proportion  to  the  com- 
pression, alike  in  degree  of 
severity  and  in  rapidity  of 
development.  It  is  important 
to  recognise  these  differences. 
The  mechanism  of  compression 
varies.  The  most  frequent  is 
the  collection  of  inflammatory 
products  outside  the  dura  mater 
and  the  thickening  of  this  mem- 
brane. Less  commonly  the  cord 
is  compressed  by  the  displace- 
ment of  the  bone,  or  by  frag- 
ments of  bone  that  are  pushed 
into  the  canal.  Often  both  these 
causes  are  influential,  as  in  the 
case  shown  in  Fig.  92,  in  which 
the  compression  (at  s)  is  between 
displaced  bone  in  front  and  in- 
flammatory products  (e)  behind. 
Probably  an  inflammatory  swell- 
ing or  abscess  of  the  bone  is 
sometimes  the  mechanism  of 
compression,  since  the  signs  of 
pressure  have  disappeared  when 
an  abscess  has  formed  outside 
the  spine,  or  even  when  defor- 
mity has  come  on — the  breaking 
down  of  the  bodies  having  relieved  the  compression  produced  by  theii 
enlargement. 


Fia.  92. — Caries  of  the  spine,  mid-dorsal 
region.  The  spinal  cord  is  much  nar- 
rowed and  discoloured  at  s  from  com- 
pression between  the  displaced  bone  and 
a  mass  of  inflammatory  products,  B, 
outside  the  dura  mater,  d  m,  the  inner 
surface  of  which  ia  normal.  (After 
Leyden.) 


OAEIES.  289 

Symptoms. — Caries  of  tte  spine  causes  symptoms  of  tliree  classes : 
(1)  those  of  the  bone  disease ;  (2)  the  effects  of  damage  to  the  nerve- 
roots;  (3)  those  dne  to  the  changes  in  the  cord  itself.  Only  the 
symptoms  due  to  the  damage  to  nerves  and  cord  come  within  the 
special  province  of  this  book,  but  the  bone  symptoms  are  cf  much 
importance,  as  on  them  the  diagnosis  of  the  cause  of  the  nervous 
symptoms  often  depends.  It  is  this  which  gives  the  subject  its  high 
medical  importance,  and  brings  the  symptoms  and  recognition  of  bone 
disease  into  the  province  of  practical  medicine. 

Symptoms  of  Bone  Disease. — The  first  is  pain  in  the  spine,  chiefly 
felt  at  the  affected  spot,  increased  by  movement,  and  especially  by 
pressure  on  the  bone.  The  local  tenderness  is  a  very  important  sign. 
It  is  elicited  both  by  direct  pressure  on  the  spines  and  by  lateral 
pressure ;  if  they  are  grasped  and  pressed  to  one  side  considerable  pain 
is  usually  produced.  Nevertheless  tenderness  is  occasionally  absent, 
not  only  in  cases  of  slight  character  and  doubtful  nature,  but  also  in 
those  in  which  paraplegia  has  come  on  some  time  after  local  curi^ature. 
The  increase  of  pain  by  movement  is  greatest  when  the  disease  is  in 
the  more  mobile  parts  of  the  vertebral  column,  especially  when  it  is  in 
the  cervical  region.  Movement  of  the  head  occasions  pain,  and  there 
is  an  instinctive  fixation  of  the  head,  which  is  sometimes  inclined  to 
right  or  left,  less  commonly  backwards.  It  may  thus  produce  the 
aspect  of  torticollis,  which  differs  from  that  due  to  muscular  contrac- 
tion in  that  the  sterno-mastoid  is  tense  on  the  side  towards  which  the 
head  is  turned,  the  muscle  being  simply  stretched  by  the  deviation  of 
the  head. 

The  deformity  of  the  spine  is  a  later  symptom  than  the  tenderness, 
and  usually  comes  on  gradually.  Its  characters  have  been  already 
described.  It  is  often  absent  when  the  disease  is  in  the  cervical 
region.  In  this  part  there  is  usually  another  symptom — ^thickening 
of  the  tissues  about  the  spine,  which  is  rare  La  other  parts.  In  any 
region  an  abscess  may  form  in  the  neighbourhood  of  the  disease. 
Those  which  come  backwards,  or  descend  to  the  groin  by  the  psoas 
muscle,  can  be  recognised  externally.  Those  that  form  in  front  of  the 
diseased  vertebrae  may  give  rise  to  symptoms  that  are  puzzling,  if  the 
existence  of  spinal  caries  is  not  known.  Thus  a  retro-pharyngeal 
abscess  may  cause  difficulty  of  deglutition,  and  one  in  the  dorsal 
region  may  cause  symptoms  of  oesophageal  obstruction. 

Increased  pain  and  tenderness  in  the  vertebral  column  often  precede 
the  indications  of  damage  to  its  contents.  The  symptoms  due  to  in- 
terference with  the  nerve-roots  are  very  variable,  and  may  be  severe  or 
absent ;  usually  they  are  moderate  in  degree,  and  consist  of  pain  on  move- 
ment along  the  course  and  in  the  distribution  of  the  nerves  that  emerge 
at  the  affected  region,  often  hypersesthesia  of  corresponding  extent, 
sometimes  with  spots  of  anaesthesia.  There  is  also  muscular  weakness 
and  sometimes  muscular  wasting.  These  sjrmptoms  are  more  fully 
described  in  the  chapter  on  Compression.  Here  it  may  suffice  to 
VOL.  I.  19 


290  DISEASES   OF   THE    SPINE. 

say  tliat  they  are  conspicuous  only  in  tlie  minority  of  tlie  cases,  and 
chiefly  occur  when  there  is  pachymeningitis.  The  motor  symptoms 
are  most  marked  when  the  disease  is  in  the  cervical  region,  and  the 
nerve-roots  for  the  muscles  of  the  arms  are  damaged,  since  slight  and 
limited  impairment  is  more  readily  recognised  in  the  arms  than  in  the 
trunk.  If  the  disease  is  in  the  highest  part  of  this  region,  the  paias 
may  be  referred  to  the  occiput.  Herpes  zoster  has  been  occasionally 
met  with  along  the  course  of  the  irritated  nerves.  Spasmodic  contrac- 
tion in  the  muscles  supplied  by  the  affected  roots  is  extremely  rare  in 
cases  of  caries.  Reflex  action  is  abolished  in  the  affected  parts,  and 
the  change  in  the  superficial  reflexes  of  the  trunk  sometimes  gives 
important  diagnostic  information.  Disease  of  the  lower  cervical  roots 
may  cause  symptoms  of  deranged  action  of  the  sympathetic  on  that 
side  of  the  head,  occasionally  shown  in  the  pupil,  more  often  in  the 
vessels.  I  have  seen  persistent  sweating  on  one  half  of  the  forehead 
from  this  cause. 

The  symptoms  of  interference  with  the  functions  of  the  spinal  cord 
itself  are  chiefly  due  to  impairment  of  its  conducting  power,  causing 
paralysis  below  the  lesion.  As  the  disease  is  most  frequently  in  some 
part  of  the  dorsal  region,  paralysis  of  the  legs  is  the  common  effect. 
But  the  symptoms  vary  in  their  characters,  according  not  only  to  the 
position  of  the  disease,  but  also  to  the  immediate  process  of  damage 
to  the  cord.  The  signs  of  caries  may  have  existed  for  years  before 
paralysis  comes  on.  Angular  curvature  may  even  come  on  in  early 
childhood,  and  paralysis  not  till  adult  life  More  commonly  the 
interval  between  the  two  is  not  long,  varying  from  a  few  months  to 
one  or  two  years,  but  either  may  first  be  conspicuous.  The  cases  are 
numerous  in  which  caries  is  not  suspected  until  the  paralysis  leads  to 
an  examination  of  the  spine,  and  it  is  not  uncommon  for  the  signs  of 
bone  disease  to  remain  equivocal  for  some  time  after  paraplegia  has 
developed. 

The  cord  symptoms  vary  much  in  their  mode  of  onset.  Usually 
there  is  no  exciting  cause,  but  sometimes  a  strain  of  the  back  or 
exposui-e  to  cold  seems  to  excite  a  change  in  the  condition  of  the 
bones,  in  consequence  of  which  the  cord  suffers.  It  is  easy  to  conceive 
that,  when  the  conditions  are  favorable,  a  very  slight  strain  may  be 
effective,  even  as  slight  as  the  sneezing  in  the  case  mentioned  below. 
When  the  cord  symptoms  have  commenced,  they  may  develop  quickly 
or  slowly.  As  instances  of  chronic  onset  may  be  mentioned  cases  in 
which  the  symptoms  reached  a  considerable  degree  of  intensity  at  the 
end  of  nine  months,  four  months,  and  two  months,  after  their  com- 
mencement. But  the  onset  is  sometimes  much  more  rapid;  in  one 
■case  there  was  complete  paraplegia  at  the  end  of  three  weeks.  Some- 
times it  is  still  more  acute,  and  in  such  cases,  when  there  is  no  corre- 
sponding change  in  the  bone,  acute  myelitis  set  up  by  slight  pressure 
is  probably  the  mechanism  by  which  the  cord  suffers.  Thus,  in  one 
patient,  a  child  of  three,  sUght  weakness  existed  for  three  weeks,  anr" 


CARIES.  291 

then  the  power  of  standing  was  lost  in  a  single  niglit.  In  another 
child,  aged  eight,  who  had  presented  for  two  yeats  indications  of 
disease  of  the  cervical  vertebrae,  the  power  of  moving  the  legs  was  lost 
in  the  course  of  twenty -four  hours;  during  the  second  day  the  left 
arm  became  paralysed,  and,  at  the  end  of  a  week,  the  right  arm. 
Very  rarely  the  onset  is  instantaneous,  probably  from  sudden  dis- 
placement. A  child  with  angular  curvature  was  walking  across  the 
room,  when  she  fell,  and  on  being  lifted  up  the  legs  were  powerless. 
The  more  rapidly  compression  occurs,  the  slighter  is  the  amount 
necessary  to  abolish  conduction. 

Both  legs  usually  suffer  together;  rarely  one  is  paralysed  before 
the  other ;  very  rarely  one  leg  suffers  much  and  the  other  little  or  not 
at  all.  Thus  in  one  case  angular  curvature  developed  in  childhood ; 
at  sixteen  there  was  an  attack  of  weakness  in  the  legs,  which  passed 
away  at  the  end  of  three  weeks.  At  17-j  the  patient  sprained  his  back; 
pain  in  it  followed,  and  six  weeks  later  the  right  leg  gradually  became 
weak,  and  a  year  afterwards  presented  intense  spastic  paralysis,  the 
left  leg  being  very  little  affected.  He  idtimately  recovered.  An 
instance  in  which  one  leg  became  affected  before  the  other  is  that  of 
a  woman,  forty-five  years  of  age,  who  suffered  from  pain  in  the  spine, 
and  one  day,  when  walking,  sneezed  violently  three  times,  and  imme- 
diately felt  "  pins  and  needles  "  about  the  right  knee,  and  presently  in 
the  foot.  The  leg  became  almost  powerless  during  the  next  three  days. 
A  fortnight  afterwards  she  felt  similar  tingling  in  the  left  knee,  and 
at  the  end  of  another  week  in  the  foot,  and  during  the  next  three 
weeks  this  also  lost  power,  so  that  at  the  end  of  six  weeks  from  the 
onset  both  legs  were  motionless.  The  diagnosis  was  verified  on  her 
death,  six  months  later. 

The  characters  of  the  paralysis  in  relation  to  the  seat  of  the  disease 
are  described  in  the  chapter  on  Compression.  In  the  most  frequent 
cases  the  dorsal  cord  is  damaged,  and  whenever  the  compression  is 
above  the  lumbar  enlargement  the  condition  of  the  legs  is  that  of 
"  spastic  paraplegia."  If  it  is  situated  in  the  cervical  region  there 
may  be  muscular  atrophy  in  the  arms,  sometimes  palsy  without 
atrophy,  according  to  the  seat  of  the  disease.  When,  as  is  com- 
monly the  case,  the  arms  suffer  from  damage  to  the  nerve-roots, 
they  suffer  before  the  legs,  but  if  the  disease  is  so  high  up  that  the 
arms  suffer  from  the  damage  to  the  cord,  the  legs  may  be  paralysed 
before  the  arms,  as  in  the  case  of  the  child  mentioned  above.  In  this 
case  the  muscles  of  the  shoulder  were  wasted,  the  disease  being  at  the 
level  of  their  nerves,  while  the  forearm  muscles  were  not  wasted.  In 
this  case,  as  in  others  of  similar  seat,  the  diaphragm  was  paralysed. 
The  muscular  part  of  the  spinal  accessory  may  be  involved,  and  the 
power  of  supporting  the  head  may  be  almost  lost.  Very  rarely,  from 
the  disease  of  the  highest  cervical  vertebrae,  other  nerves  of  the  medulla 
are  implicated,  especially  the  hypoglossal  and  the  fibres  of  the  spinal 
accessory  for  the  palate. 


292  DISEASES    OF   THE    SPINE. 

Sensory  symptoms  due  to  the  disease  of  tlie  cord  are  less  com.moii 
than  motor  palsy.  The  onset  of  paraplegia  may  be  preceded  by 
subjective  sensations  in  the  legs.  Dull  aching  pain  in  them  is  also 
not  uncommon.  Often  there  is  no  loss  of  sensation ;  in  other  cases 
there  are  various  degrees  of  loss.  Touch  or  pain  may  be  impaired 
alone,  or  there  may  be  absolute  loss  of  sensibility  up  to  the  level  of 
the  lesion.  Partial  loss  is  more  common.  Keflex  action  in  all  forms 
is  excessive  (unless  the  disease  involves  the  lumbar  enlargement),  and 
great  excess  of  the  superficial  reflex  action  is  a  common  and  important 
feature.  The  legs  are  often  cold,  and  sometimes  perspire  continually. 
The  sphincters  are  often  affected,  sometimes  early,  but  they  may  escape 
even  where  there  is  complete  motor  palsy  of  the  legs. 

Complications. — Among  common  complications  are  bedsores,  cystitis, 
various  secondary  effects  of  the  bone  disease,  such  as  abscess,  local 
scrofulpus  disease  elsewhere,  and  general  tuberculosis.  Tubercular 
tumours  in  the  brain  occasionally  coincide  with  the  caries.  In  very 
severe  cases  peculiar  secondary  mischief  has  occurred  in  the  spinal 
cord,  and  has  run  an  independent  course,  giving  rise  to  very  anomaloua 
symptoms.*  Thus  a  descending  myelitis  may  invade  the  lumbar 
enlargement  in  its  entirety  and  abolish  its  central  and  reflex  func- 
tions, causing  rapid  wasting  of  the  muscles  and  acute  trophic  changes 
in  the  skin.  Inflammation  may  ascend  the  pyramidal  tracts  and  thus 
paralyse  the  arms — an  instance  of  the  strange  limitation  of  inflamma- 
tion to  functional  tracts  when  it  passes  in  a  direction  opposite  to 
secondary  degeneration.  Ascending  degeneration  of  the  posterior 
median  columns  may  spread  to  the  postero-external  columns,  and 
cause  symptoms  of  ataxy  by  invading  root-fibres  at  a  higher  level. 
Ataxy  may  come  on  as  the  power  returns,  when  the  disease  is  in  the 
dorsal  region,  probably  by  damage  to  the  path  from  the  muscles  to 
the  cerebellum.  Lastly,  myelitis  may  occur  in  disseminated  foci  in 
various  parts  of  the  cord,  and  even  in  the  medulla,  giving  rise  to 
scattered  symptoms  of  anomalous  character. 

Course. — The  bone  disease  may  heal,  imion  occurring  between  the 
altered  tissues,  or  it  may  persist  with  continued  formation  of  pus,  or 
may  become  quiescent  with  occasional  periods  of  renewed  activiiy. 
The  cord  mischief  is  influenced  by  the  state  of  the  bone  disease, 
although  its  progress  may  be  to  some  extent  independent.  Thus 
inflammation  in  the  cord,  in  excess  of  the  compression,  may  subside 
in  spite  of  the  continuance  of  the  bone  mischief.  Pressure  on  the  cord 
may  be  relieved,  although  the  bone  disease  continues,  and  even  some- 
times as  a  result  of  the  increased  breaking  down  of  bone  and  exit  of 
pus  by  another  channel.  A  case  is  mentioned  below  in  which  para- 
plegia passed  away  as  angular  curvature  developed.  Hence  there  is 
no  strict  correspondence  between  the  course  of  the  bone  mischief  and 
that  of  the  cord  disease.  In  some  cases  the  paralysis,  motor  and 
sensory,  persists.  More  often  the  sensory  loss  passes  away,  while 
•  See  Charcot,  •Le9ons  sur  les  Mai.  da  Syst.  Nerv.,'  torn.  ii. 


OAEIES.  293 

motor  paralysis  remains,  usually  as  spastic  paraplegia,  and,  in  severe 
cases,  flexor  spasm  comes  on  with  muscular  contractions.  Life  may 
be  prolonged  in  that  condition  for  years,  but  often,  in  such  cases, 
bedsores  form,  or  cystitis  leads  to  kidney  disease,  or  other  tubercular 
disease  develops  and  leads  to  death,  or  the  lessened  respiratory  power 
renders  an  attack  of  bronchitis  fatal.  In  many  cases,  again,  the 
paralysis  gradually  passes  away  even  when  the  compression  continues, 
and  the  cord  is  found  considerably  narrowed  if  the  patient  dies  from 
some  other  cause.  It  is  possible  that  such  compression  is  sometimes 
produced  slowly  without  impairment  of  conduction.  In  children  re- 
covery occurs  far  more  readily  than  in  adults.  Even  in  adults,  how- 
'  ever,  recovery  may  occur  from  palsy  that  has  la.sted  for  more  than  a 
year,  with  all  the  signs  of  descending  degeneration  in  the  cord, 
amounting  to  severe  spastic  paraplegia.  Usually  rest  on  the  back 
or  mechanical  supports  are  necessary  to  secure  recovery,  but  it 
occasionally  occurs  without  these  mea.sures.  A  youth  acquired  angular 
curvature  at  sixteen ;  at  twenty  paraplegia  came  on  slowly,  and  pro- 
gressed with  some  variations  during  the  next  seven  years.  There 
was  then  absolute  motor  palsy  in  the  legs,  and  sensation  was  lessened. 
He  refused  to  rest,  and  continued  to  follow  his  occupation,  which  was 
however  a  restful  one,  that  of  a  tailor ;  he  took  cod-liver  oil  and  iron, 
and  gradually  regained  useful  power,  so  as  to  be  able  to  walk  about. 
Such  a  case,  however,  is  exceptional. 

Eelapses  sometimes  occur  in  cases  that  improve,  although  they  are 
certainly  not  nearly  so  frequent  as  might  be  expected  from  the  nature , 
of  the  disease.  In  the  majority  of  cases  recovery,  once  obtained,  is 
permanent.  In  a  minority  the  paralysis  returns  when  some  exciting 
cause  renews  activity  in  the  bone  disease.  The  tendency  to  relapse  and 
the  possibiUty  of  repeated  recovery  are  very  strikingly  shown  by  the 
following  case.  In  a  girl  of  fifteen,  paraplegia  developed  during  nine 
months,  slowly  at  first,  more  rapidly  towards  the  end  of  that  time. 
She  came  under  my  care  six  months  later,  having  been  unable  even 
to  move  her  legs  for  that  time.  Bone  disease  had  not  been  previously 
suspected,  but  there  was  slight  tenderness  and  enough  lateral  irregu- 
larity of  the  lower  dorsal  spines  to  show  the  nature  of  the  case.  Rest 
in  bed  and  tonics  were  soon  followed  by  imj)rovement ;  in  six  weeks 
she  could  stand,  and  in  four  months  was  able  to  walk  well.  As  she 
gained  powef,  angular  curvature  came  on,  prominence  of  the  seventh 
and  eighth  dorsal  spines.  Five  months  after  her  discharge  she  fell 
and  struck  her  back ;  the  ciu'vature  increased,  and  her  legs  gradually 
became  weak  again.  Seven  months  after  the  fall  she  was  readmitted, 
unable  to  stand,  although  the  paralysis  was  not  absolute.  There  was 
foot-clonus  on  each  side.  Sensibility  was  lessened  below  the  ensiform 
cartilage.  Rest  on  the  back  was  again  followed  by  slow  improvement. 
In  three  months  she  could  just  walk.  She  was  then  suspended,  and 
encased  in  plaster  of  Paris.  At  the  end  of  another  month  she  could 
walk  about  the  room,  and  no  clonus  could  be  obtained.     She  was  soon 


294.  DISEASES    OP  THE    SPINE. 

afterwards  discharged,  and  lier  progress  continued,  so  that,  at  the  end 
of  nine  months,  she  could  walk  five  miles,  and  there  was  no  trace  of 
clonus,  although  there  was  still  some  excess  of  the  knee-jerk.  She 
soon  afterwards  married,  and  bore  a  child,  which  died  two  years  and  a 
half  after  her  discharge.  She  caught  cold  at  the  funeral,  and  a  fort- 
night later  again  began  to  lose  power ;  in  six  weeks  the  legs  were 
almost  motionless,  with  marked  foot-clonus ;  sensation  to  touch  was 
lost  up  to  the  umbilicus,  that  of  pain  being  preserved.  Neither  rest 
nor  encasement  caused  any  improvement.  After  some  months  sulphide 
of  calcium  was  given,  and  in  a  few  days  power  began  to  return ;  in  a 
month  she  could  take  a  few  steps,  and  in  four  months  she  could  walk 
about  the  ward  without  difficulty.  She  made  another  good  recovery. 
Some  years  later,  however,  paralysis  again  came  on,  and  this  attack 
proved  permanent. 

Several  cases  have  come  under  my  notice  in  which  the  subjects  of 
caries  in  early  life,  which  healed  without  damaging  the  cord,  have  at 
some  period  in  adult  life  presented  the  symptoms  of  primary  lateral 
sclerosis — simple  spastic  paraplegia,  without  any  root  symptoms  or 
evidence  of  renewed  activity  of  the  bone  disease.  If  there  is  a  con- 
nection between  the  two,  it  is  probable  that  the  cord  has  suffered 
compression  so  slowly  that  its  functions  have  not  been  interfered  with, 
but,  nevertheless,  the  vitality  of  the  pyramidal  fibres  has  been  rendered 
less  enduring. 

It  is  important  to  remember  that  the  deviation  is  often  lateral,* 
and  the  chief  difficulty  is  due  to  the  fact  that  a  slight  lateral  deviation 
or  slight  prominence  is  not  unusual  in  normal  spines.  Hence  it  is 
important  for  the  student  to  make  himself  familiar  with  the  degrees 
of  deviation  that  occur  in  health.  At  the  same  time  it  must  not  be 
forgotten  that  a  deviation  not  greater  than  occurs  in  health  may  be 
due  to  disease.  If  it  coincides  with  distinct  tenderness,  and  especially 
also  with  the  position  of  root  symptoms,  it  may  be  accepted  as  evidence 
of  disease,  probable  or  certain,  according  to  the  character  of  the 
symptoms. 

The  pathology  of  the  affection  only  concerns  us  so  far  as  it  relates 
to  the  effect  on  the  spinal  cord,  and  this  is  considered  in  the  chapter 
on  Compression. 

Diagnosis. — When  clear  indications  of  caries  precede  the  paralysis, 
the  nature  of  the  case  can  hardly  be  mistaken.  The  obvious  inference, 
that  the  affection  of  the  cord  is  secondary  to  that  of  the  bone,  is 
scarcely  ever  wrong.  When  the  two  develop  together,  mistakes  in 
diagnosis  are  often  made,  but  are  usually  due  to  the  want  of  repeated 
examinations  of  the  spinal  column.  It  is  when  the  root  or  cord 
symptoms  precede  distinct  evidence  of  bone  disease,  and  when  the 
latter  is  so  slight  as  to  be  equivocal,  that  the  chief  real  difficulty  in 

*  To  discover  lateral  deviation  it  is  well  to  make  an  ink-dot  on  the  skin  over  the 
middle  of  the  tip  of  each  spine,  care  being  taken  that  the  skin  ia  not  stretched  to 
one  side.     The  ink-dots  may  be  copied  on  tracing-paper. 


CAETBS.  295 

diagnosis  occurs;  the  affection  is  apt  to  be  mistaken  for  a  primary 
disease  of  the  oord  or  its  membranes — a  transverse  myelitis  when  the 
dorsal  region  is  affected,  a  progressive  muscular  atrophy,  or  primary 
pachymeningitis,  when  the  disease  is  in  the  cervical  region.  A  correct 
diagnosis  can  only  be  made  in  these  cases  by  recognising  the  signifi- 
cance of  the  slight  bone  symptoms  that  are  always  present,  the  deep 
tenderness,  and  often  slight  irregularity.  Even  slight  irregularity 
derives  significance  from  tenderness,  limited  in  extent  and  correspond- 
ing in  position  to  the  deviation.  The  irregularity  may  be  a,bsent  at 
first,  and  then  its  development  is  doubly  significant.  An  observed 
increase  in  the  amoujat  of  displacement  gives  significance  to  even  a 
slight  irregularity. 

The  early  excess  of  the  cutaneous  reflex  action  (from  the  sole,  for 
instance) ,  while  not  conclusive,  adds  weight  to  the  other  symptoms  of 
bone  disease.  It  may  be  very  marked  even  while  the  patient  is  able 
to  walk  about.  Distinct  root  pains  are  always  of  great  significance, 
and  this  is  increased  by  tlie  detection  of  spots  of  anaesthesia  along 
their  course.  All  these  symptoms  derive  additional  weight  from  their 
coincidence  in  level  with  the  spinal  irregularity. 

It  is  this  which  enables  the  pains  to  be  distinguished  from  the  con- 
dition for  which  they  are  most  frequently  mistaken — a  trunk  neuralgia. 
This  error  is  especially  common  when  the  pain  is  chiefly  unilateral.  In 
all  such  cases  the  spine  should  be  carefully  examined,  and  any  tender- 
ness of  the  bone  at  the  level  of  the  pain  should  excite  suspicion.  I 
have  also  known  unilateral  abdominal  pain  due  to  caries  to  be  mis- 
taken for  that  of  renal  calculus. 

When  damage  to  the  cervical  roots  causes  muscular  wasting  in  the 
arms,  the  case  may  be  mistaken  for  one  of  progressive  muscular 
atrophy,  but  differs  m  the  distribution  of  the  wasting,  in  the  pains, 
and  the  impan-ment  of  sensation.  These  occur  in  primary  cervical 
pachymeningitis,  which  is  distinguished  by  the  absence  of  the  signs 
of  bone  disease,  and.  in  the  wider  extent  through  which  the  root 
symptoms  extend. 

In  the  first  half  of  life  the  recognition  of  bone  disease  is  practically 
tantamount  to  the  recognition  of  caries.  In  the  second  half,  however, 
the  relative  infrequency  of  caries,  the  greater  frequency  of  growths  in 
the  bone,  and  the  occurrence  of  eroding  aneurisms,  introduce  a  fresh 
diagnostic  problem.  The  absence  of  any  other  indication  of  a  tumour 
or  an  aneurism  is  the  first  distinction ;  and  the  second  is  the  fact  that 
in  both  these  diseases  the  root  pains  commonly  reach  a  degree  of 
severity  scarcely  ever  attained  in  caries,  a  ad  are  especially  increased 
by  movement. 

When  there  are  merely  tenderness  of  the  spine  and  slight  weakness 
of  the  legs,  the  question  may  arise  whether  there  is  organic  disease,  or 
merely  the  condition  termed  "  spinal  irritation,"  or  mere  functional 
pain  and  tenderness  and  weakness  of  the  legs.  In  these  cases  the 
tenderness  is  usually  found,  over  a  considerable  area  of  the  spinal 


296  DISEASES    OF  THE    SPINE. 

column,  witli  more  than  one  point  of  special  intensity ;  it  is  superficial 
as  well  as  deep,  and  may  change  its  seat ;  there  are  no  root  pains  or 
spots  of  anaesthesia.  There  is  more  danger  that  caries  of  the  spine  in 
a  young  woman  may  be  passed  as  hysterical  paraplegia  than  of  the 
opposite  error.  Especially  when  the  subjects  of  caries  present  distinct 
symptoms  of  hysteria,  there  is  risk,  as  experience  shows,  that  un- 
equivocal symptoms  of  caries  may  be  overlooked.  Some  other 
diagnostic  indications  are  mentioned  in  the  chapter  on  Compres- 
sion. 

Peognosis. — Our  ignorance  of  the  precise  character  of  the  morbid 
process  which  is  damagiag  the  cord  renders  the  prognosis,  ia  every 
case  of  caries,  a  matter  of  much  uncertainty.  Nevertheless  there  is 
no  disease  of  the  cord  in  which  symptoms  of  equal  gravity  so  often 
pass  away.  The  cases  are  few,  therefore,  in  which  hope  is  unjustified, 
but  they  are  equally  few  in  which  we  are  warranted  in  a  confident 
expectation  of  recovery.  In  childhood  the  prospects  of  recovery  are 
certainly  better  than  in  adidt  life,  and  they  are  least  in  declining 
years.  Damage  to  the  cord  between  the  enlargements  is  less  serious 
than  when  these  are  affected,  because  the  strong  tendency  to  trophic 
changes  constitutes  a  grave  danger  when  the  lumbar  enlargement  ia 
diseased,  and  the  diminished  breathing  power  an  equally  serious 
danger  in  disease  of  the  cerAdcal  enlargement,  especially  when  this  is 
high  enough  to  entail  the  additional  danger  of  paralysis  of  the 
diaphragm. 

But  how  perilous  a  condition  may  be  recovered  from  is  shown  by 
the  fact  that  the  child  mentioned  on  p.  291  had  paralysis  of  all  four 
limbs,  the  diaphragm,  and  weakening  of  the  intercostals,  and  yet 
recovered.  Still  more  striking  is  a  case  narrated  by  Dr.  Buzzard,  in 
which  disease  in  the  region  of  the  third  cervical  vertebra  caused 
almost  complete  palsy  of  arms,  legs,  intercostals,  and  diaphragm, 
respiration  being  carried  on  by  the  accessory  muscles  of  the  neck» 
Yet  the  child  recovered  in  spite  of  the  occurrence  of  an  attack  of 
pneumonia  when  the  paralysis  was  at  its  height.  A  girl  of  thirteen, 
whose  cervical  caries  was  accompanied  by  all  the  symj)toms  of  cere- 
bellar tubercle,  recovered.  All  these,  it  will  be  noted,  were  children. 
Neither  rapidity  nor  slowness  of  onset  affords  any  guide  to  prognosis, 
nor  does  the  relative  order  of  paralysis  and  curvature,  or  the  degree 
of  palsy.  Severe  spastic  paraplegia  may  pass  away  entirely,  provided 
it  remains  extensor  in  character.  The  prognosis  is  perhaps  a  little 
better  when  there  is  no  loss  of  sensation,  since  this  proves  that  the 
damage  to  the  cord  is  moderate  in  degree ;  but  even  complete  anaes- 
thesia does  not  preclude  recovery,  as  the  cases  mentioned  show.  The 
danger  to  life  is  dependent  in  considerable  degree  on  the  evidence  that 
the  scrofulous  or  tubercular  tendency  is  active  elsewhere,  and  also  on 
any  difficiilty  in  securing  proper  treatment.  The  prognosis  is,  more- 
over, still  in  a  transitional  state  on  account  of  the  uncertainty  regard- 
inof  the  range  of  successful  surgical  treatment. 


CARIES.  297 

Treatment. — The  first  and  cHef  element  in  tlie  treatment  of 
paralysis  is  tliat  of  tlie  bone  disease  wliicli  causes  it,  and  for  fuU 
•details  of  this  the  reader  is  referred  to  treatises  on  surgery.  It 
includes  both  the  older  means  of  securing  an  arrest  of  the  morbid 
process,  and  the  still  recent  measures  of  operative  treatment.  If 
the  bone  disease  heals,  the  spinal  cord,  in  most  cases,  will  recover. 
The  two  most  potent  therapeutic  agents  are  persistent  recumbency 
and  tonics,  especially  cod-liver  oil  and  iron.  In  the  cases  that 
have  done  best,  these,  and  these  alone,  have  been  the  effective 
agents.  Eest  should  be  maintained  for  months.  The  posture  that 
answers  best  is  upon  the  back,  on  account  of  the  greater  ease  with 
which  immobility  of  the  bone  is  secured  and  maintained,  and  the 
great  importance  of  this  element  in  treatment;  this  position  is  also 
the  most  comfortable,  and  local  pain  probably  interferes  with  the  sub- 
sidence of  local  inflammation.  If  no  improvement  occurs  after  some 
months'  rest,  suspension  may  be  tried.  It  is  said  to  be  sometimes 
attended  by  instant  improvement,  especially  in  children;  but  this 
result  is  rare,  and  can  only  occur  in  the  cases,  not  very  common,  in 
which  displaced  bone  compresses  the  cord.  In  cases  in  which  there  is 
reason  to  suspect  that  this  is  the  cause  of  paralysis,  it  may  be  well  to 
see  the  effect  of  suspension  before  rest  is  commenced.  The  moulded 
jacket  is  an  inefficient  substitute  for  rest,  and  an  unnecessary  con- 
<;omitant — to  be  adopted  only  when  rest  cannot  be  secured,  or  after 
rest  alone  has  apparently  done  all  that  it  can  achieve.  If,  for  instance, 
power  gradually  returns  diu-ing  rest,  but  the  improvement  after  a  time 
ceases,  and  the  bone  disease  seems  inactive,  the  patient  may  be  sus- 
pended, encased,  and  then  permitted  to  stand  and  walk ;  improvement 
may  be  renewed  and  power  may  rapidly  increase.  In  caries  of  the 
mobile  cervical  spine,  even  during  the  period  of  rest,  fixation  of  the 
head  is  necessary.  Extension  of  the  spine  in  the  recumbent  posture 
has  also  been  employed,  sometimes  with  apparent  benefit.  The  head 
of  the  patient  is  fastened  to  the  head  of  the  bed  by  an  elastic  band 
attached  to  a  strap  passing  beneath  the  occipital  protuberance  and 
round  the  patient's  head.  A  weight  of  from  4  to  12,  or  even  20  lbs., 
is  fastened  to  a  band  passing  round  the  patient  above  the  hips.  Con- 
tinuous extension  is  thus  obtained.*  More  recently  sudden  forcible 
extension  of  the  spine  and  immediate  reduction  of  the  deformity  has 
been  recommended  as  a  mode  of  treatment  for  spinal  caries.  In  some 
cases  in  which  paraplegia  was  present,  rapid  return  of  power  in  the 
])aralysed  limbs  has  occurred  after  this  treatment ;  but  it  is  obvious 
that  such  a  method  has  inherent  in  it  certain  dangers  which  are  at 
least  minimised  in  less  heroic  treatment.f 

The  influence  of  cod-hver  oil  and  iron  is  as  marked  in  this  as  in 
other  scrofulous  diseases.     They  constitute  an  indispensable  adjunct 

*  Flemin<r, '  Lancet,'  April  27th,  1889. 

t  Discussion  at  Clinical  Society  of  London,  *  Lancet,*  Nov.  12th  and  26th,  1897. 


298  DISEASES  OF  THE  SPINE. 

to  rest,  and  may  be  effective  even  alone,  as  is  shown  by  tbe  case  men- 
tioned on  p.  293.     But  rest  should  always  be  secured  if  possible. 

Counter-irritation,  opposite  the  seat  of  caries,  has  sometimes 
appeared  to  do  good,  perhaps  acting  especially  on  the  process  of 
myelitis  which  attends  compression.  The  actual  cautery,  in  mild 
form,  is  that  which  has  been  most  frequently  found  useful.  Instances 
of  speedy  improvement  are  on  record,  but  it  is  not  always  effective, 
nor  can  indications  for  its  use  be  laid  down.  Sulphide  of  calcium, 
■which  is  said  to  influence  scrofulous  processes,  deserves  further  trial, 
although  I  have  not  found  it  so  apparently  effective  in  any  other  case 
than  that  mentioned  on  p.  294.  Benefit  can  only  be  expected  from  its 
use  when,  as  is  often  the  case,  inflammatory  products  constitute  the 
compressing  agent.  Extreme  care  in  general  management,  to  avoid 
bedsores,  bladder  trouble,  and  bronchitis,  are  of  great  importance. 
In  manv  cases  there  is  a  tendency  to  improvement  after  a  time, 
especially  when  the  bone  disease  is  stationary,  or  the  cord  has  been 
inflamed,  and  in  these  the  mere  maintenance  of  life  may  result  in 
recovery  of  strength.  When  power  has  returned,  but  the  use  of  the 
limbs  is  restrained  by  the  muscular  contractions  which  come  on  dtiring 
the  paralysis,  the  ability  to  stand  and  walk  may  be  quickly  restored 
by  tenotomy,  and  more  slowly  by  the  use  of  extending  splints,  &c. 
Electricity  is  useful  only  to  maintain  the  nutrition  of  the  musclea 
when  these  are  wasting,  in  consequence  of  damage  to  their  nerves. 

The  behef  expressed  in  the  first  edition  of  this  work,  that  the  cases 
are  numerous  in  which  relief  to  the  compression  may  be  afforded  by 
the  surgeon,  has  been  fully  realised.  The  path  was,  indeed,  being 
then  opened  up  by  McEwen  of  Glasgow,  who  has  shown,  with  others, 
especially  Yictor  Horsley,  how  safe  and  successful  the  operative 
treatment  is  in  properly  selected  cases ;  and  these,  indeed,  include 
a  large  proportion  of  the  cases  in  which  other  measures  have  failed 
to  give  relief  and  must  probably  have  remained  unsuccessful.  The 
operation  is  contra- indicated  in  cases  of  rapidly  advancing  general 
tuberculosis  (to  which  most  deaths  after  operation  have  been  due, 
and  also,  as  a  rule,  in  children,  in  whom  other  measures  are,  as  has 
already  been  stated,  usually  successful)  When  the  indirect  con- 
sequences of  the  disease  have  greatly  reduced  the  patient's  strength^ 
the  question  of  an  operation  may  still  be  entertained  if  it  is  a  dis- 
tinct alternative  to  a  speedy  death,  provided  the  peril  is  such  as  a» 
restoration  of  the  functions  of  the  cord  might  possibly  remove.  On 
the  other  hand,  the  operation  ought  not  to  be  regarded  as  the  first 
resort  in  the  majority  of  cases.  The  large  number  of  cases  that, 
recover  under  the  treatment  described  above  make  it  certain  that,  in 
most  cases,  these  measures  should  first  be  tried ;  and  this  conclusion 
is  the  clearer  since  the  duration  of  paraplegia,  for  a  year  or  more, 
seems  to  interpose  no  appreciable  barrier  either  to  recovery  or  to  the 
relief  that  can  be  given  to  the  pressure.  On  the  contrary,  indeed,  the 
prospect  of  recovery  is  better  if  the  bone  disease  has  become  quiescent,. 


GROWTHS    IN   THE    SPINE.  299 

or  tte  caries  lias  actually  healed,  processes  tliat  nmst  he  furthered  by 
the  preliminary  rest.  The  operation  of  laminectomy  has  the  best  pro- 
spect of  success  in  the  cases,  fortunately  very  numerous,  in  which  the 
products  of  inflammation  outside  the  dura  mater  compress  the  cord. 
These,  whether  caseous  material  or  a  mass  of  connective  tissue  into 
which  the  inflammatory  products  have  been  changed,  can  be  readily 
removed  without  opening  the  dura  mater.  Even  if  displaced  bone  is  the 
source  of  pressure,  the  removal  of  the  arches  affords  relief  to  it.  The 
chief  obstacle  to  success  is  the  extent  to  which  myelitis  has  proceeded 
out  of  proportion  to  the  compression.  This,  necessarily,  no  removal  of 
the  pressure  can  relieve.  In  proportion  as  the  inflammation  has  been 
acute  without  evidence  of  rapid  compression,  relief  is  unlikely.  But 
this  is  not  the  only  cause  of  a  rapid  onset,  and  a  difl&cult  question 
arises  in  such  cases,  whether  the  mechanism  is  not  such  that  an 
immediate  operation  is  justified.  It  may  be  due  to  sudden  displace- 
ment of  bone  or  to  sudden  escape  of  pus  into  the  canal,  as  well  as  to 
myelitis,  and  in  the  two  former  cases  it  is  possible  that  relief  may 
sometimes  be  given,  and  an  immediate  operation  may  be  wiser  than 
delay.  An  operation  is  suggested  whenever  a  sudden  increase  in  the 
curvature  or  severe  root-pains  coincide  with  the  acute  onset  of 
paralysis.  Without  these  symptoms,  the  rapid  onset  is  probably  due 
to  acute  myelitis,  and  then  an  operation  cannot  be  expected  to  lessen 
the  interference  with  the  conducting  fibres.  It  is  also  called  for  in 
cases  in  which  the  disease  is  high  up,  and  by  its  interference  with  the 
respiratory  mechanism  is  threatening  life.  To  justify  surgical  inter- 
ference, however,  the  existence  of  caries  must  be  beyond  doubt.  The 
operation  involves  a  small  danger  to  life,  although  it  may  not  be 
great,  and  this  fact  must  always  receive  due  consideration  in  regard 
to  the  certainty  of  diagnosis  as  well  as  the  prospect  of  relief. 


TUMOURS  AND  OTHER  DISEASES  OF  THE  SPINE. 

GrROWTHS   IN   THE    SpINAL    CoLTJMN. 

The  bones  of  the  spine  are  sometimes  the  seat  of  primary  or 
secondary  growths,  and  less  commonly  are  invaded  by  tumours 
springing  from  the  various  structures  and  even  organs  in  front  of  the 
spine.  They  may  also  undergo  changes  as  the  result  of  the  ©bscure 
condition  known  as  osteomalacia,  and  these  changes  may  occasion  pres- 
sure on  the  cord,  and  so  give  rise  to  paraplegia.*     Cancer  (scii-rhous 

*  Koppen,  *  Arch.  f.  I'sycli.,'  xxii. 


SOO  DISEASES  OF  THE   SPINE. 

and  enceplialoid)  and  sarcoma  are  tlie  most  common  forms  of  growth ; 
myxoma  has  been  also  met  witli.  Cancer  is  sometimes  secondary  to  a 
primary  grovsrtb.  elsewhere,  in  the  breast,  stomach,  &c.  A  primary 
growth  usually  begins  in  the  bodies  of  the  vertebrae,  and  spreads  from 
one  to  another.  The  bodies  may  collapse,  because  the  soft  tissue 
which  replaces  the  bone  yields  uuder  the  weight  it  has  to  bear,  and 
thus  angular  curvature  may  occux,  or  some  other  abrupt  deviation 
from  the  normal  line.  From  the  bodies  the  growth  may  extend  into 
the  lateral  processes,  enlarging  them,  and  narrowing  the  intervertebral 
foramina  through  which  the  nerves  pass.  It  may  extend  into  the 
arches,  and  even  into  the  spines,  and  may  also  grow  into  the  muscles 
and  tissues  beside  and  behind  the  vertebral  column.  The  nerve-roots 
suffer  (1)  by  pressure,  (2)  by  simjDle  inflammation,  (3)  sometimes  by 
cancerous  infiltration.  They  may  thus  be  found  reddened,  swollen  and 
soft,  or  grey  and  atrophied,  or  enlarged  and  hardened.  The  growth, 
cancer  especially,  is  apt  to  spread  in  the  adipose  tissue  between  the 
bone  and  the  dura  mater,  and  may  even  entirely  surround  the  dura 
mater  and  cord.  The  cord  may  suffer  compression,  but  this  is  less 
frequent  than  in  caries,  even  when  curvature  occurs.  On  the  other 
hand,  it  is  often  inflamed,  sometimes  acutely ;  considerable  inflamma- 
tion without  compression  is  far  more  frequent  than  in  caries.  The 
myelitis  occurs  without  any  perforation  of  the  dura  mater  by  the 
growth.  The  cause  of  this  tendency  to  acute  local  inflammation  is 
not  known;  once  excited,  it  may  spread  in  the  cord  upwards  and 
downwards.     The  growth  never  invades  the  cord  itself. 

ExioLOGT. — The  general  causes  are  the  same  as  those  of  similar 
growths  elsewhere.  Males  suffer,  however,  more  frequently  than 
females,  and  the  morbid  growths,  taken  together,  are  most  common 
between  forty  and  fifty  years  of  age.  An  injury  has  been  supposed 
sometimes  to  be  the  exciting  cause  in  this  as  in  other  situations. 

Symptoms. — There  may  be  direct  symptoms  of  the  presence  of  the 
growth — pain  in  the  spine,  usually  severe,  occasionally  absent;  local 
tenderness,  and  occasional  interference  with  movement  of  the  vertebral 
column  apart  from  pain.  A  palpable  tumour  is  never  an  early  sym- 
ptom, but  ultimately  a  deep-seated  hard  swelling  may  be  felt  on  one 
side  of  the  spine,  most  readily  and  earliest  when  the  disease  is  in  the 
cervical  region,  where  it  is  occasionally  perceptible  in  the  posterior 
triangle  of  the  neck.  The  angular  curvatui'e  which  sometimes  occurs 
does  not  differ  from  that  of  caries  in  its  external  characters,  except 
that  it  is  more  frequently  a  rounded  prominence  than  a  sharp  angle, 
and  it  may  be  accompanied  by  the  indications  of  a  tumour,  never  by 
those  of  an  abscess. 

The  affection  of  the  nerves  is  almost  invariable  in  spinal  growths, 
and  gives  rise  to  the  symptoms  that  are  the  most  distressing,  and 
also  the  earliest  and  most  constant, — radiating  pain  in  the  coui'se  of 
the  nerves  that  emerge  at  the  part,  and  due  to  their  irritation  by  pres- 
sure or  inflammation.     Such  pains  are  far  more  prominent  symptoms 


GROWTHS   IN   THE    SPINE.  301 

in  tliis  disease  than  in  caries.  At  first  slight,  they  gradually  increase 
to  extreme  intensity.  It  was  this  feature  that  led  Cruveilhier  to  call 
the  disease  paraplegia  dolorosa,  a  name  it  has  since  commonly  borne. 
The  pain  at  first  intermits,  but  subsequently  is  constant,  with 
paroxysms  of  greater  suffering  from  time  to  time.  It  is  usually  a 
sharp  and  lancinating  pain,  and  its  special  characteristic  is  the  degree 
in  which  it  is  increased  by  movement.  Even  slight  movements  of 
the  trunk  induce  the  paroxysms.  The  distribution  in  the  arms, 
trunk,  legs,  depends  on  the  seat  of  the  disease.  It  is  extremely  rare 
for  these  pains  to  be  absent,  but  they  occasionally  occur  late  instead 
of  early.*  On  the  other  hand,  they  may  precede  other  symptoms  for 
months.  Cutaneous  hypersesthesia  usually  accompanies  them,  and 
spots  of  ansesthesia  often  develop,  after  a  time,  in  the  areas  to  which 
the  fixed  pains  are  referred.  Corresponding  damage  to  the  motor 
roots  may  cause  painful  muscular  contracture  (Figs.  56  and  57, 
p.  112),  paralysis,  and  wasting.  Contractures  may  also  occur  in  the 
parts  below  the  disease,  produced  by  the  persistent  irritating  pressure 
on  the  motor  tract  in  the  cord.  Paroxysms  of  spasm  often  attend  the 
pains,  especially  in  the  abdominal  muscles  when  the  disease  is  in  the 
dorsal  region,  and  are  apparently  reflex  in  nature. 

The  damage  to  the  cord  causes  symptoms  similar  to  those  in  caries, 
and  described  more  fully  in  the  chapter  on  Compression.  The 
chief  difference  from  caries  is  the  greater  frequency  of  a  rapid  onset 
of  the  paraplegic  symptoms,  due  to  the  invasion  of  the  cord  by  acute 
inflammation.  All  power  in  the  legs  is  often  lost  in  twelve  or  twenty- 
four  hours,  and  this  when  no  curvature  has  taken  place.  Such  acute 
paraplegia  is  not  uncommonly  preceded  for  a  week  or  so  by  retention 
of  urine,  probably  indicative  of  slight  pressure  on  the  cord.  Displace- 
ment of  bone  has  also  been  known  to  cause  rapid  paralysis. f  On  the 
other  hand,  especially  in  very  slowly  growing  tumours,  the  onset  of 
the  palsy  may  be  gradual.  I  have  known  it  to  occupy  several  years 
in  reaching  a  considerable  degree.  Probably  in  these  cases  the 
mechanism  is  a  simple  slow  compression.  Thus,  compared  with  caries, 
the  onset  is  more  often  rapid,  and  occasionally  much  more  deliberate. 
The  characters  of  the  resulting  paralysis  are,  as  a  rule,  similar  to 
those  in  caries,  but  are  more  frequently  modified  by  the  spread  of 
inflammation,  immediate  or  subsequent.  Hence  the  central  and 
reflex  functions  of  the  lumbar  enlargement,  although  the  bone  disease 
is  some  distance  above  it,  may  be  lost  at  the  onset,  or  they  may  be  at 
first  normal  or  excessive,  and  afterwards  lost.  In  a  man  with  a  growth 
in  the  mid-dorsal  region,  paraplegia  came  on  rapidly,  evidently  from 
myelitis,  and  was  followed  by  foot-clonus,  &c.  But  a  few  weeks  later 
the  clonus  suddenly  ceased,  the  muscles  became  toneless,  with  loss  of 
faradic  irritability,  and  the  skin  began  to  slough;  the  inflammation 

*  The  pains  succeeded  curvature  in  a  case  described  by  L.  Humphry  ('Lancet,' 
1884, 5,  p.  15). 
•f"  Humphry,  loCt  cit. 


802  DISEASES  OP  THE    SPINE. 

had  spread  down  into  the  lumbar  enlargement.  In  many  cases,  how- 
ever, there  is  no  descending  inflammation,  and  the  reflex  actions  are 
in  persistent  excess,  so  that  spastic  paraplegia  results.  Sensation  is 
lost  rather  more  frequently  than  in  caries.  Other  symptoms  are  the 
same  as  in  compression  from  any  cause.  The  course  of  the  disease  is 
from  its  nature  progressive;  occasionally,  however,  some  improve- 
ment succeeds  a  rapid  development  of  palsy,  and  is  due  to  the 
partial  recovery  of  a  cord  damaged  disproportionately  by  inflammation. 
It  is  seldom,  however,  that,  life  is  prolonged  for  a  sufficient  length  of 
time  for  this  to  occur.  Much  more  rarely  the  pains  lessen,  although 
the  growth  spreads — perhaps  from  destruction  of  irritated  nerves. 

The  duration  of  the  disease  varies  according  to  the  nature  of  the 
growth.  In  cancer  it  is  to  be  measured  by  months.  In  slowly 
growing  tumours  the  symptoms  may  last  for  years.  Death  may  be 
due  to  bedsores,  &c.,  to  cystitis  and  kidney  disease,  to  growths  else- 
where, or  the  patient  may  be  simply  worn  out  by  the  prolonged 
agony.  In  one  curious  case  a  growth  from  an  intervertebral  cartilage 
caused  characteristic  symptoms  for  thirteen  years,  and  then  death  by 
meningeal  haemorrhage.* 

Diagnosis. — The  recognition  of  the  disease  is  only  a  matter  of  cer- 
tainty when  signs  of  a  tumour  are  present,  but  the  probability  almost 
amounts  to  certainty  when  such  symptoms  as  those  mentioned  follow  a 
primary  growth  elsewhere.  Such  pain  as  that  above  described,  occur- 
ring in  a  person  from  whom  a  malignant  tumour  has  been  removed, 
should  always  be  regarded  with  grave  suspicion,  and  the  grounds  for 
this  are  not  lessened  by  either  an  interval  of  several  years  since  the 
removal  of  the  tumour,  or  by  the  completeness  with  which  this  was 
effected.  Especially  is  this  true  of  cases  of  mammary  cancer.  The 
pains  shoidd  lead  to  careful  and  repeated  examination  of  the  spine  and 
a  search  for  any  abrupt  deviation,  of  any  kind,  and  in  any  part  of  the 
vertebral  column ;  while  the  cervical  spine  should  be  examined  at  the 
sides  and  from  the  posterior  triangle  of  the  neck,  the  character  of  the 
bony  prominences  on  the  two  sides  being  minutely  compared.  Indi- 
cations of  a  growth  may  often  be  thus  discovered  long  before  they  are 
obtrusive.  It  is  also  important  to  remember  the  fact  that  acute 
transverse  myelitis  may  be  an  early  effect  of  the  disease,  and  if  this 
lesion,  in  such  a  subject,  coincides  with  marked  local  tenderness,  the 
probability  of  secondary  growth  in  the  bone  is  very  great;  it  is 
rendered  still  greater  by  preceding  pains,  and  certain  by  coincident 
deformity. 

It  must  be  remembered  that  similar  root  symptoms  are  sometimes 
due  to  an  aortic  aneurism,  or  to  a  growth  in  front  of  the  vertebral 
column,  commencing,  for  instance,  in  the  glands,  and  irritating  the 
nerves  as  they  emerge  from  the  intervertebral  foramina. 

From  intercostal  neuralgia,  the  influence  of  movement  on  the  pain 
is  usually  a  sufficient  distinction,  even  when  cord  symptoms  are 
*  Boiveit.  'Arch,  de  Phj's.,'  1887,  No.  8. 


GROWTHS   IN  THE    SPINE.  803 

absent.  The  commonly  bilateral  character  of  the  pain  is  a  further 
difference.  The  symptoms  of  a  growth  in  the  lower  cervical  region 
are  sometimes  very  closely  simulated  by  symptoms  which,  from  their 
character  and  course,  are  probably  due  to  radicular  neuritis.  There 
are  severe  root-pains,  referred  to  one  arm  (or  less  commonly  to  both 
arms),  increased  by  movement  and  without  marked  tenderness  of  the 
plexus  of  nerve-trunks.  The  subjects  are  also  in  the  later  period 
of  life.  The  distinction  rests  on  the  absence  of  the  signs  of  a  growth, 
on  the  slighter  effect  of  motion  on  the  pains,  on  the  absence  of  anj 
progressive  tendency,  and  on  the  common  presence  of  a  history  of 
gout,  and  especially  on  the  absence  of  any  symptoms  of  compression 
of  the  cord  even  after  the  nerve-roots  have  suffered  for  some  months. 
Sometimes  it  is  necessary  to  wait  and  watch  the  course  of  the  sym- 
ptoms. It  is  necessary  to  be  very  careful  in  assessing  the  value  of 
slight  irregularities ;  the  vertebra  prominens  is  apt  to  be  thought  to 
project  too  much  even  for  its  designation,  and  a  deep  bilateral  swelling 
is  readily  assumed,  under  normal  conditions,  when  the  head  is  bent 
forward. 

The  chief  difficulty  in  diagnosis  is  the  distinction  from  caries 
when  distinct  evidence  of  a  growth  is  absent.  In  the  first  half 
of  life  caries  would  alone  be  thought  of  in  such  a  case,  but  in 
the  second  half  the  two  diseases  are  about  equally  frequent.  One 
distinction — suggestive,  not  absolute — is  the  intensity  of  the  pain 
in  tumour,  taken  in  conjunction  with  its  great  increase  when  the 
patient  laoves.  It  is  tmie  that  the  root-pains  of  caries  are  said  to  be 
Bometimes  most  severe,  but  such  severity  is  not  frequent  enough  to 
destroy  the  significance  of  intensity.  I  have  not,  for  instance,  seen  a 
single  case  of  caries  (of  a  large  number)  in  which  the  pain  was  com- 
parable to  that  in  most  of  the  cases  of  growth  that  have  come  under 
my  observation.  Therefore,  while  absence  of  pain  is  of  slighter  dia- 
gnostic value  (in  favour  of  caries),  great  severity,  and  agonising 
increase  by  movement,  are  strongly  in  favour  of  vertebral  growth. 
In  each  disease  there  may  be  angular  curvature ;  but  this,  in  growths, 
is  usually  soon  succeeded  by  other  signs  of  tumour.  In  caries  these 
signs  are  absent,  and  an  abscess  often  develops.  A  history  or  indica- 
tion of  tubercle  is  almost  conclusive  evidence  of  caries.  These  points 
will,  I  believe,  avail  for  the  distinction  in  most  cases.  In  a  few  it  is 
necessary  to  wait  and  watch  before  an  opinion  can  be  formed. 

Among  other  diseases  with  which  these  growths  may  be  confounded 
IS  the  dorsal  form  of  tabes,  in  which  severe  radiating  pains  occur  in  the 
trunk  and  not  in  the  legs.  But  the  wide  extent  of  the  root  symptoms, 
the  slight  effect  of  movement,  and  the  fact  that  the  knee-jerk  is  lost, 
suffice  for  the  distinction.  I'here  is  also  usually  more  extensive  dis- 
turbance of  sensation  on  the  trunk.  The  distinction  from  tumours  of 
the  spinal  cord  and  membranes  is  considered  in  a  later  chapter. 

Peog-nosis. — The  prognosis  scarcely  requires  formulating.  The 
chief  differences  are  in  the  time  that  life  is  likely  to  last.     The  pains 


804  DISRASES  OP  THE  SPINE. 

usuallj  persist,  in  spite  of  the  progress  of  the  disease,  although  there 
is  a  hare  possibility  of  their  subsidence.  The  chance  of  any  return  of 
power  in  the  paralysed  part  is  small,  although  not  quite  absent  if  the 
palsy  develops  in  a  manner  to  suggest  a  secondary  myelitis,  and  the 
progress  of  the  growth  itself  is  slow. 

Tbeatment. — ^Possibly  in  a  few  cases  a  growth  may  be  so  placed  as 
to  be  removed,  and  an  exploratory  operation  would  be  justifiable  ia 
any  case  that  might  prove  suitable ;  otherwise  treatment  must  neces- 
sarily be  confined  to  the  relief  of  pain,  and  to  the  avoidance  of  bed- 
sores and  other  results  of  the  cord  disease.  Morphia  is  alone  powerful 
for  the  rehef  of  pain,  but  unhappily  the  dose  has  to  be  quickly 
increased,  and  the  power  of  the  drug  is  lessened  by  custom.  It 
becomes  a  race  between  dose  and  pain,  in  which,  if  life  lasts  long,  the 
pain  not  uncommonly  gets  in  front  of  the  narcotic.  Cocaine,  however, 
affords  some  relief  in  many  cases,  and  may,  with  other  anodynes,  at 
least  prolong  the  influence  of  moderate  doses  of  morphia. 

Vertebrai,  Exostoses. 

Exostoses  sometimes  grow  from  the  bodies  of  the  vertebrae  into  the 
spinal  canal,  and  may  compress  the  cord  or  nerves.  They  are,  how- 
ever, exceedingly  rare.  The  symptoms  may  be  those  of  slow  compres- 
sion of  the  cord,  or  of  irritation,  expressed  chiefly  by  pain.  They 
usually  resemble  those  of  a  tumour  of  the  cord  or  membranes  rather 
than  of  the  bones,  but  the  pain  is  occasionally  much  increased  by  move- 
ment. Their  chief  characteristic  is  extreme  chronicity.  In  one  case 
the  patient  suffered  frequent  intense  paroxysms  of  pain  in  the  right 
groia,  which  had  persisted  for  two  years,  with  occasional  intervals  of 
freedom.  There  was  some  weakness  of  the  legs,  but  no  considerable 
paralysis.  Ten  years  before,  he  had  had  some  loss  of  sensibility  in 
each  thigh,  which  had  passed  away.  An  intra- spinal  tumour  was 
diagnosed ;  the  post-mortem  examination  revealed  exostoses  from  the 
bodies  of  the  ninth  and  tenth  dorsal  vertebrae,  slightly  compressing 
the  cord.  Although  extreme  chronicity  may  raise  a  suspicion  of 
exostosis,  it  is  doubtful  whether  a  confident  diagnosis  is  ever  justified 
except  in  the  cases  in  which  there  are  similar  exostoses  elsewhere. 
This  rare  indication  existed  in  a  patient,  under  the  care  of  my  col- 
league Dr.  Barlow,  who  presented  multiple  exostoses,  and  paraplegia 
of  gradual  onset,  which  was  supposed  during  life,  and  found  after 
death,  to  be  due  to  a  similar  exostosis  within  the  spinal  canal.  It  had 
sprung  from  one  of  the  lumbar  vertebrae,  and  had  compressed  the 
nerves  of  the  cauda  equina. 

Exostoses  constitute  a  more  promising  field  for  the  surgeon  than 
other  kinds  of  vertebral  tumour.  Many  of  them  are  so  placed  that 
their  removal  is  feasible.  If  situated  in  front  of  the  cord,  the 
division  of  some  nerve-roots,  at  least  in  the  dorsal  region,  might 
permit  access  to  the  growth. 


EROSION  BY  ANEURISM.  305 


Syphilitic  Disease. 


Syphilitic  caries  of  the  bodies  of  the  vertebrae  is  a  rare  variety,  the 
symptoms  of  which  do  not  differ  from  those  of  the  scrofulous  form. 
It  has  been  observed  ia  the  cervical  region,  secondary  to  deep  syphilitic 
ulceration  of  the  pharynx.  Nodes  of  the  vertebrae,  vsdthin  the  canal, 
are  occasionally  presumed  to  exist  and  to  compress  the  cord,  but  I  do 
not  know  of  any  pathological  observation  confirming  the  assumption, 
and  it  is  probable  that  most  of  the  supposed  instances  have  been  cases 
of  syphilitic  gummata  in  the  meninges.  Deep-seated  thickening  of 
the  tissues  about  the  cervical  vertebrae  sometimes  occurs  in  syphilitic 
subjects.  It  may  develop  on  one  side  or  both,  and  is  apparently  due 
to  a  syphilitic  cellulitis.  The  swelling  may  be  felt  either  on  each  side 
and  behind  the  upper  cervical  spine,  or  deep  in  the  posterior  triangle 
of  the  neck.  It  may  damage  the  nerves  before  they  enter  the  brachial 
plexus,  causing  a  defined  palsy,  as  in  one  case  of  the  lower  arm 
muscles.  Movements  of  the  neck  may  be  interfered  with,  and  irrita- 
tion of  the  nerves  may  cause  neuralgia-like  pain,  generally  felt  down 
the  arm,  and  often  very  severe.  Except  by  the  absence  of  nodula- 
tion,  it  is  scarcely  to  be  distinguished  from  a  deep-seated  growth. 
The  spmal  cord  does  not  usually  suffer.  All  the  symptoms  pass  away 
onder  antisyjjhilitic  treatment.  In  one  late  case,  however,  iodide 
had  no  iafluence,  although  mercury  quickly  cured. 

Eeosion  by  Anetjeism. 

Bones,  like  other  structures,  may  atrophy  and  waste  before  the 
pressure  of  an  aneurism,  and  the  bodies  of  the  dorsal,  or  rarely  of 
the  lumbar  vertebrae  may  be  thus  eroded  by  aneurisms  of  the  aorta. 
The  pressure  and  absorption  take  place  from  the  left  side.  Two  or 
three  vertebrae  usually  suffer,  and  the  bodies  more  than  the  inter- 
vertebral cartilages.  The  periosteum  becomes  thickened,  and  may 
resist  the  pressure  and  to  some  extent  protect  the  cord.  Sometimes, 
however,  the  cord  becomes  compressed,  or  the  periosteum  may  come 
to  form  part  of  the  wall  of  the  aneurism,  and  may  give  way  before 
the  blood-pressure,  so  that  rupture  occurs  into  the  spinal  canal. 

The  symptoms  vary  much.  Pain  along  the  nerve-roots  is  usually 
severe,  but  this  may  attend  aneurisms  that  merely  compress  the  nerves 
after  their  emergence,  and  do  not  damage  the  bone.  The  process  of 
erosion  is  usually  attended  by  severe  pain  ia  the  spine.  But  in  the 
case  of  a  patient  under  the  care  of  Dr.  HughHngs  Jackson,  an  aneurism 
of  the  arch  of  the  aorta  had  eroded  the  spinal  column,  caused  pressure 
myelitis,  which  gave  rise  to  complete  paraplegia  and  paralysis  of  the 
sphincters,  without  causing  any  pain.  The  patient  died  of  kidney 
disease  secondary  to  cystitis.  When  the  cord  is  reached,  compres- 
sion causes  the  usual  paraplegic  symptoms,  of  slow  or  rapid  onset. 
Eupture  into  the  canal  is  attended  by  sudden  complete  paraplegia  and 
VOL.  I.  20 


306  DISEASES    OF    THE    SPINE. 

death,  either  immediate,  or  in  tlie  course  of  a  few  hours,  from  ascending 
paralysis,  due  to  the  haemorrhage  around  the  spinal  cord. 

The  diagnosis  is  scarcely  possible  unless  other  indications  of 
aneurism  are  detected,  since  the  symptoms  closely  resemble  those  of  a 
growth  in  the  bone.  The  nature  of  the  disease  may,  however,  be 
suspected  if  such  symptoms  as  have  been  described  are  succeeded  by 
sudden  paraplegia  followed  quickly  by  ascending  paralysis. 


Hydatid  Disease. 

Hydatid  cysts  sometimes  develop  in  the  loose  adipose  tissue 
between  the  dura  mater  and  the  bone,  and  it  is  beheved  that  they 
sometimes  form  in  the  substance  of  the  bones  themselves.  About 
a  dozen  cases  have  been  collected  by  Ley  den.*  As  the  cyst  grows, 
the  bone  of  the  arches  sometimes  becomes  atrophied  by  pressure,  and 
the  cyst  may  develop  outside  the  canal,  so  that  there  is  a  double 
cyst,  outside  and  inside,  connected  by  a  narrower  part.  Occasionally 
the  cyst  develops  also  in  front  of  the  spine,  in  the  thorax  or  abdomen. 
The  internal  cyst  necessarily  compresses  the  spinal  cord,  which  often 
also  becomes  inflamed.  The  usual  paraplegic  symptoms  develop,  both 
motion  and  sensation  being  usually  lost,  Eadiating  pains  along  the 
nerve-roots  are  frequent.  The  symptoms,  in  themselves,  resemble  too 
closely  those  caused  by  other  diseases  of  the  spinal  column  to  permit 
a  diagnosis  to  be  made,  unless  similar  disease  elsewhere  suggests  the 
nature  of  the  spinal  lesion,  or  unless  the  cyst  can  be  felt  in  the  back, 
when  a  puncture  may  prove  its  nature.  All  the  cases  hitherto  recorded 
have  terminated  fatally,  but  if  removal  can  be  effected  a  cure  may  be 
possible.  In  one  case,  however,  a  girl  aged  twenty-two,  in  whom  a 
small  tumour  beside  the  last  dorsal  and  first  lumbar  spines  was  opened 
(on  account  of  complete  palsy  of  legs,  bladder,  and  rectum),  a  con- 
tinuous discharge  of  echiuococcus  cysts  occurred,  more  than  a  hundred 
escapiug  in  the  course  of  four  months,  when  the  patient  died  in  conse- 
quence of  ascending  damage  to  the  cord.  The  tumour  had  been  noticed 
for  eight  years  before  death,  during  four  of  which  it  produced  sym- 
ptoms of  irritation  of  the  nerves,  and  finally  prominence  of  the 
vertebral  spines  developed.f  But  in  a  case  under  my  care,  with  sym- 
ptoms of  an  intra-spinal  tumour  in  the  lumbar  region,  Mr.  Horsley 
trephined  the  spine  and  found  a  quantity  of  hydatid  cysts  compressing 
the  cord.  These  were  removed,  and  the  patient  recovered,  but  the 
damage  was  too  great  to  allow  the  recovery  of  the  extensive  atrophic 
paralysis  produced  in  the  right  thigh  and  hip  muscles. 

Numerous  small  cysticercal  vesicles  have  also  been  occasionally 
found  within  the  dura-matral  sheath.  In  one  case  several  such 
vesicles  were  attached  to  nerve-roots,  one  to  each,  in  the  lumbar 
region,  while  a  considerable  number  on  the  cervical  enlargement  were 

•  'Klinik  der  Riickenm.  Krankh.,'  Band  i. 

t  Pedjkow,  «Med.  Obos.'  (Russ.),  xxviiij  and  '  Cent.  f.  Nerv,'  xii,  271. 


VEETEBRAL    AETHRITIS.  807 

beneatli  tlie  pia  araclinoid.*  Sucli  cysts  may  compress  the  cord, 
producing  paraplegia,  and  even  death.  As  in  other  forms  of  com- 
pression, the  paraplegia  maj  come  on  suddenly.f 


Diseases  op  the  Aetictjlations. 

Lateral  Curvattjke  of  the  Spine  seldom  affects  the  functions 
of  the  cord.  Even  when  slight  compression  has  occurred,  the  slow- 
ness of  its  production  has  apparently  prevented  interference  with 
function.  In  very  rare  cases  some  weakness  of  the  legs  has  been 
present,  possibly,  but  not  certainly,  the  result  of  the  curvature.  Occa- 
sionally the  intervertebral  foramina  have  been  narrowed,  and  the 
pressure  on  the  nerves  has  caused  radiating  neuralgic  pains. 

Vertebral  Arthritis. — In  chronic  rheumatoid  arthritis  the  inter- 
vertebral articulations  are  sometimes  involved.  The  symptoms  are 
local  pain,  tenderness,  and  limitation  of  movement,  which  may  go 
on  to  absolute  fixation,  especially  in  the  cervical  region,  if  anchy- 
losis occurs.  I  have  known  the  whole  neck  to  be  rigid  from  this 
cause,  in  a  case  in  which  also  the  movement  of  the  lower  jaw  was 
much  reduced.  More  frequently,  movement  is  restricted  by  pain 
before  the  mechanical  limit  is  reached.  Sometimes  an  occasional 
clicking  sound  occurs.  Enlargement  of  the  ends  of  th<^  bones  may 
occur,  and,  in  thin  persons,  may  even  be  felt.  The  paiu  is  increased 
by  movement,  and  often  by  changes  in  the  weather,  aud  by  fatigue. 
It  is  often  felt  through  a  considerable  extent  of  the  spine,  and  may 
extend  to  the  back  of  the  head.  The  cord  is  scarcely  ever  compressed* 
but  the  narrowing  of  the  foramina  may  damage  the  nerve-roots.  The 
articular  processes,  it  will  be  remembered,  form  one  side  of  the  inter- 
vertebral foramina,  which  are  thus  encroached  on  by  any  enlargement 
of  the  bones.  The  nerves  may  thus  be  compressed  and  irritated,  and 
radiating  pains  are  produced,  sometimes  even  a  descending  neuritis. 
The  nerve-trunks  in  the  limbs  may  then  become  tender  as  well  as 
painful ;  muscular  atrophy  may  even  occur,  and,  when  the  disease  is 
in  the  cervical  region,  there  may  be  symptoms  of  disturbance  of  the 
sympathetic.  Spasmodic  torticollis  is  said  to  have  been  caused  by 
this  disease  in  the  cervical  region.  The  atlo-occipital  articulation 
has  been  affected  alone,  or  with  the  adjacent  vertebrae,  and  peculiar 
interference  with  the  movement  of  the  head  has  resulted.  Sometimes 
the  foramen  magnum  is  narrowed,  or  the  odontoid  process  projects 
into  the  canal.  Symptoms  of  damage  to  the  nerves  of  the  medulla 
have  also  been  produced  (Solbrig),  In  the  dorsal  region  the  sym- 
ptoms produced  are  slighter  in  degree,  but  similar  in  general  cha- 

*  Hirt,  'Berl.  kl.  Wochenschr.,'  1887,  No.  8.  The  relation  of  the  symptoms  to 
the  parasitic  disease  is  doubtful  in  a  degree  that  makes  it  useless  to  mention  them. 

t  As  in  a  case  recorded  by  Wiegand  ('  Warsaw  Med.  Ob.|*  and  *  Cent.  f.  JNerv.,* 
1888,  665). 


808  SPINAL    MEMBRANES. 

racter,  altliougli  the  local  symptoms  usually  preponderate  over  tliose 
of  irritation  of  tlie  nerves.     The  whole  spine  may  even  become  rigid. 

The  degree  in  which  the  morbid  process  causes  symptoms  varies, 
however,  very  much  in  different  cases,  and  it  has  occasionally  been 
found  out  at  the  post-mortem  examination  without  being  suspected 
during  life.  It  is  usually  associated  with  distinct  evidence  of  a  rheu- 
matic diathesis,  and  commonly  with  obvious  arthritis  elsewhere. 
This  is  the  most  important  diagnostic  indication  as  to  the  cause  of 
the  spinal  symptoms,  but  it  must  be  remembered  that  the  vertebral 
affection  sometimes  exists  alone,  and  it  may  cause  symptoms  during 
many  years,  often  when  their  nature  is  unsuspected.  It  occurs  in  both 
sexes,  and  is  met  with  in  young  women,  in  whom  the  symptoms  are 
often  ascribed  to  hysteria.  The  treatment  is  the  same  as  that  of 
the  general  disease,  nerve  symptoms  alone  needing  special  treatment, 
such  as  is  described  in  the  section  on  diseases  of  the  nerves.  The 
most  important  point  in  regard  to  the  malady  is  its  diagnosis,  because 
distant  nerve-pain  is  liable  to  be  regarded  as  unconnected  with  local 
disease,  or  the  disease  to  be  thought  far  more  serious  than  it  really  is, 
and  especially  to  be  mistaken  for  chronic  meningitis  or  malignant 
disease,  or  even  tubercular  mischief. 


DISEASES  OF  THE  MEMBBANES  OF  TEE  SFINAL  COED. 

The  general  arrangement  of  the  membranes  of  the  spinal  cord  has 
been  ali-eady  mentioned  (p.  199).  The  pia  mater  closely  invests  the 
cord,  while  the  arachnoid  forms  a  loose  sheath  around  it,  and  the  two 
membranes  are  connected  by  trabeculae  and  membranous  expansions  of 
fine  connective  tissue,  which  occupy  the  "  subarachnoid  space."  Each 
surface  of  the  dura  mater  is  covered  by  a  layer  of  epithehum.  The 
outer  sheaths  of  the  nerves  are  continuous  with  this  membrane ;  the 
connective  tissue,  within  the  outer  sheath,  is  continuous  with  both  the 
pia  mater  and  arachnoid.  The  blood-vessels  of  the  cord  ramify,  as  we 
have  seen,  in  the  pia  mater,  and  the  lymphatic  canals  of  the  two  are 
continuous.  Most  of  the  cerebro- spinal  fluid  within  the  vertebral 
canal  is  contained  in  the  subarachnoid  space,  but  there  is  a  little  fluid 
between  the  dura  mater  and  the  arachnoid.  Both  membi'anes  are 
supplied  with  nerves,  but  those  of  the  pia  mater  are  the  more  abun- 
dant. 

The  morbid  processes  that  involve  the  membranes  are  chiefly  three 
— growth,  hsemorrhages,  and  inflammation.  Tumours  of  the  mem- 
branes may  be  conveniently  considered  in  connection  with  those  of  the 
spinal  cord.    Inflammation  and  haemorrhage  will  be  here  described. 


EXTEENAL   MENINGITIS.  809 


SPINAL    MENINGITIS:    INFLAMMATION    OF   THE 
MEMBRANES    OF   THE    SPINAL    CORD. 

Inflammation  of  the  membranes  may  be  acute  or  clironic,  and  may 
begin,  and  affect  chiefly,  the  dura  mater  (pachymeningitis,  i.  e.  inflam- 
mation of  the  firmer  membrane),  or  the  pia  mater  (lejjt omening itis, 
inflammation  of  the  softer  membrane).  The  arachnoid  usually  suffers 
with  the  pia  mater,  but  is  sometimes  the  seat  of  inflammation  that 
affects  the  pia  mater  but  little,  a  form  that  has  been  termed  arachnitis. 
Acute  inflammation,  beginning  in  one  membrane,  usually  spreads  to 
the  others.  Chi'onic  inflammation  may  remain  limited  to  one  mem- 
brane, dura  mater  or  pia  mater. 

All  forms  of  acute  inflammation,  wherever  they  begin,  cause  similar 
symptoms.  It  is  only  when  the  inflammation  is  chronic  that  the 
symptoms  may  differ  sufficiently  to  allow  of  a  precise  diagnosis. 

Spinal  meningitis  may  be  conveniently  divided  into  two  forms, 
which  depend  on  the  situation  of  the  inflammation  in  regard  to  the 
dura  mater  :  (1)  that  which  begins  outside  this  membrane,  and  some- 
times arises  by  extension  from  some  adjacent  focus  of  inflammation 
— external  meningitis;  (2)  that  which  begins  within  the  dura-matral 
sheath,  and  is  often  primary — internal  meningitis. 


EXTERNAL  MENINGITIS, 

External  meningitis  is  thus  an  inflammation  of  the  dura  mater.  It 
has  been  termed  external  pachymeningitis,*  peripachymeningitis,  and 
perimeningitis.  The  inflammation  involves  not  only  the  membrane, 
but  also  the  connective  tissue  outside  it,  in  which  are  the  venous 
plexuses  and  adipose  tissue.  This  connective  tissue  may  be  inflamed 
before  the  membrane  itself.  The  morbid  process  usually  remains 
limited  to  the  outer  surface  of  the  dura  mater,  even  when  the  inflam- 
mation is  acute  and  intense,  passing  through  it  only  in  rare  cases. 

Cattses. — External  meningitis  is  generally  due  to  extension  from 
some  contiguous  disease,  especially  of  the  bones  of  the  spine,  caries  of 
which  is  the  most  frequent  cause  of  the  affection.  It  results  also  from 
other  forms  of  bone  disease,  and  a  syphilitic  inflammation  is  probably, 
in  rare  cases,  associated  with  syphilitic  disease  of  the  vertebrae.  Such 
secondary  external  meningitis  is  generally  limited  in  extent  and 
chronic  in  course.     Deep  sacral  bedsores,  however,  sometimes  cause 

•  Pachymeningitis  interna  hsemorrhagica  has  also  been  described, — an  inflam- 
mation affecting  the  inner  surface  of  the  dura  mater,  giving  rise  to  a  fibrinous 
membrane  studded  with  minute  haemorrhages.  It  is  found  in  general  paralysis, 
and  abo  associated  with  alcoholism,  with  cerebral  tubercular  meningitis,  and  as  a 
result  oi:  injury.  But  in  such  conditions  the  pia  arachnoid  is  also  usually  involved 
(see  Internal  Meningitis). 


'SIO  SPINAL   MEMBRANES. 

an  acute  inflammation,  whieli  may  spread  widely.  An  ascending 
neuritis  lias  been  supposed  in  some  cases  to  set  up  local  inflammation. 
Suppuration  adjacent  to  the  vertebral  column  is  sometimes  associated 
witb  a  general  acute  purulent  meningitis,  and  has  generally  been 
regarded  as  the  cause  of  the  latter;  but  careful  observations  have 
rendered  it  probable  that  in  most  cases  the  external  suppuration  has 
been  the  consequence,  and  not  the  cause,  of  the  meningitis.  It  is 
certain  that  acute  general  external  meningitis  occurs  as  a  primary 
disease,  and  may  run  an  intensely  rapid  course  with  profuse  suppura- 
tion between  the  membrane  and  the  bone.  The  subjects  have  gene- 
rally been  young,  ill-nourished  adults,  and  when  any  exciting  cause 
has  been  traced,  this  has  usually  been  exposure  to  cold.  In  some 
cases  the  pus  has  worked  its  way  backwards  between  the  vertebral 
arches  (sometimes  through  the  foramina),  and  has  spread  among  the 
muscles  of  the  back,  sometimes  forming  also  collections  at  one  or 
more  spots,  either  at  the  back  or  in  front  of  the  spine, — under  the 
pleura,  for  instance,  or  behind  the  peritoneum.  Such  local  collec- 
tions of  pus  were  also  formerly  regarded  as  the  cause  of  the  menin- 
gitis. It  is  still  possible  that  this  mechanism  is  effective  in  some 
instances,  as  when  a  retro-pharyngeal  abscess  is  followed  by  the 
meningeal  affection ;  but  it  is  certain  that  in  many  cases  the  actual 
relation  is  the  reverse,  and  that  the  external  suppuration,  in  acute 
cases,  is  often  secondary.*  Hence  it  is  probable  that  this  is  true  of 
all  cases  in  which  the  evidence  of  primary  external  disease  is  not  con- 
clusive. At  the  same  time  further  observations  are  needed  to  decide 
the  point. 

Pathological  Anatomy. — The  inflammation  is  sometimes  simple, 
but  much  more  frequently  purulent  or  semi-purulent.  If  simple,  the 
dura  mater  may  be  merely  reddened  and  opaque,  with  a  little  lymph 
on  the  surface ;  when  purulent,  the  surface  is  covered  by  a  layer  of 
pus.  In  the  more  common  semi-purulent  form,  such  as  is  common  in 
caries  of  the  spine,  a  layer  of  inflammatory  products  covers  the  outer 
surface  of  the  dura  mater,  soft,  semi-caseous  or  firm  caseous  material, 
sometimes  with  liquid  pus  here  and  there  in  the  firmer  substance. 
Often  the  inflammatory  products  undergo  development  into  fibroid 
tissue,  a  mass  of  which  then  lies  between  the  dura  mater  and  the 
bones.  Such  a  layer,  caseous  or  fibroid,  may  sometimes  be  half  an 
inch  thick  (see  Pig.  92,  p.  288).  It  may  either  surround  the  dura 
mater,  or  be  chiefly  on  one  side.  Sometimes  there  is  an  irregular 
nodular  thickening  of  the  outer  layer  of  the  membrane  (Pig.  93).  In 
most  cases  the  inner  surface  is  normal,  even  when  the  cord  itself  is 
inflamed.  Occasionally  the  inner  surface  of  the  dura  mater  is  also 
inflamed,  and  then  the  internal  membranes  are  involved  and  adhe- 
sions form  between  them. 

In  all  forms  of  external  inflammation,  the  fat  outside  the  dura 

*  Two  instructive  cases  are  recorded  by  Dr.  W.  H.  Spencer, '  Lancet,'  1879,  vol.  i, 
and  Dr.  Maguire,  ib.,  1888,  vol.  ii. 


EXTERNAL    MENINGITIS.  311 

mater  quickly  become.'  absorbed,  and  the  membrane  may  ultimately 
become  adlaerent  to  the  bone,  or  connected  with  it  by  intervening 
fibrous  tissue  of  inflammatory  origin. 

Tbe  vertical  extent  oi  the  disease  varies  much;  when  secondary  to 
caries  the  inflammation  is  usually  limited  to 
the  neighbourhood  of  the  bone  disease.     When 
acute  and  general,  as  in  the  primary  form,  the 
space  between  the  membrane  and  the  bone  is 
fiUed  with  pus  through  the  whole  length  of  the 
canal.     Occasionally  the  pus  only  lies  behind 
the  membrane,  between  it  and  the  arches  of 
the  vertebrae,  apparently  determined  in  posi-     .^ 
tion  by  gravitation.      It  cer.ses  in  the  upper     / 
cervical  region,  whore   the   dura  mater  is  in     \ 
closer  contact  with  the  bone,  its  ascent  being     , 
perhaps  also  hindered  by  gravitation   as   the     I 
patient  Hes  with  the  head  raised.     As  already 
stated,  pus  is  often  found  among  the  muscles 
of    the    back    (having    escaped    between    the 
arches),  and  sometimes  collections  are  found 
in  front  of  the  spine.     The  amount  outside  is 
no  doubt  increased  rapidly  by  the  local  suppu- 
ration excited  by  the  presence  of  the  pus.     In 
these  cases  the  membrane  itself  is  swollen,  and 
has   a   sodden   aspect,  resembling   a  piece   oi' 
wash-leather,  being  softened  in  proportion  to 
the  duration  of  the  affection.     The  ianer  sur- 
face is  usually  normal,  and  the  pia  mater  is 
either  unchanged  or  merely  congested.  ^    ^,  , 

Symptoms, — In  different  cases  of  external  t,     „„     ^^  ^    ^'      ", 

.  .      ,  ITT  Fig.  93. — External  pachy- 

meningitis the  symptoms  vary  much,  and  they  meningitis  j  nodular 
are  usually  compUcated  with  those  of  the  thickening  of  outer 
cause  of  the  inflammation.  Those  that  occur  ^Jf^^  ^^  the  sphie/^  ^^ 
in   caries    have   been   already  described.      In 

most  acute  cases  the  symptoms  are  nearly  the  same  as  those  of 
internal  meningitis,  although  they  are  subject  to  greater  variations, 
and  more  frequently  have  unusual  and  anomalous  manifestations, 
presently  to  be  described.  The  chief  symptoms  are  pain  in  the 
back,  often  referred  to  the  loins,  increased  by  movement,  accom- 
panied by  stiffness  of  the  muscles  of  the  spine,  severe  root-pains,  and 
cutaneous  hypersesthesia.  The  pains  are  usually  associated  with 
spasm  ;  the  hypersesthesia  may  be  followed  by  anaesthesia,  and  some- 
times by  paralysis  of  the  muscles  suppHed  by  the  irritated  and 
damaged  nerves.  When  there  is  a  rapid  formation  of  pus,  the  func- 
tions of  the  cord  itself  are  rapidly  interfered  with  in  consequence  of 
its  compression,  and  the  symptoms  of  irritation  are  to  a  large  extent 
replaced  by  those  of  paralysis,  beginning  in  the  legs  and  ascending. 


312  SPINAL    MEMBRANES. 

The  motor  palsy  is  accompanied  by  flaccidity  of  tlie  muscles  and  loss 
of  all  reflex  actions.  Sensation  may  also  be  lost,  or  may  be  preserved 
or  changed.  The  sphincters  are  usually  paralysed.  Bedsores  form 
rapidly  if  life  is  sufficiently  prolonged.  A  peculiar  lividity  of  the  skin 
has  been  sometimes  noted.  In  these  acute  cases  there  is  much 
cox.stitutional  disturbance,  rigors,  profuse  sweating,  and  high  fever,  in 
one  case  amounting  to  110°  (Maguire). 

In  local  secondary  forms  the  symptoms  differ  in  character  according 
to  the  acuteness  of  the  inflammation,  and  in  distribution  according  to 
its  seat.  Although,  as  a  rule,  pain  is  considerable,  other  signs  of 
irritation,  stiffness  and  muscular  spasm,  are  generally  inconspicuous, 
and  the  symptoms  resemble,  and  are  merged  in,  those  due  to  the 
disease  by  vfhich  the  meningitis  is  produced. 

Diagnosis. — The  most  important  indications  of  the  clironic  form 
are  the  vertebral  pain  and  the  radiating  pains,  with  other  symptoms 
of  irritation  of  the  nerve-roots,  combined  with  the  signs  of  pressure 
on  the  cord.  These  symptoms  are  seldom  distinct  from  those  of  the 
cause  of  the  external  inflammation ;  the  meningitis  is  indeed  the  chief 
mechanism  by  which  the  local  disease  causes  its  nerve  manifestations. 

The  acute  form  causes  symptoms  that  have  much  in  common  with 
those  of  internal  meningitis.  It  is  indeed  doubtful  whether  the 
diagnosis  can  be  made  from  the  character  of  the  nerve  symptoms 
alone.  The  special  indication  of  the  seat  of  the  process  is  furnished 
by  evidence  of  its  extension  to  or  from  the  structures  outside  the 
spine.  If  such  acute  symptoms  are  secondary  to  external  disease  that 
clearly  preceded  the  meningeal  inflammation,  this  may  safely  be 
assumed  to  be  outside  the  dura  mater.  In  a  case  of  apparently 
primary  meningitis  a  careful  watch  shovild  be  kept  on  the  tissues  of 
the  back;  any  sign  of  deep  oedema  among  the  muscles  beside  the 
vertebral  column,  in  such  a  case,  is  probable  evidence  of  commencing 
purulent  inflammation  extending  from  within,  and  the  development  of 
acute  local  inflammation  in  either  the  pleura,  posterior  mediastinum, 
back  of  the  abdomen,  or  behind  the  pharynx,  has  the  same  signifi- 
cance. It  is  important  that  the  nature  of  such  symptoms  should  be 
kept  in  mind,  as  otherwise  the  occurrence  increases  the  perplexing 
character  of  the  case.  It  is  probable  that  the  nature  of  many  cases, 
which  remain  undecided  during  life,  would  be  rendered  clear  by 
repeated  careful  observation. 

Prognosis. — The  acute  affection  is  exceedingly  grave.  Eecorded 
cases  have  ended  in  death,  but  this  termination  has  been  a  condition 
of  the  diagnosis ;  and  it  is  possible  that  some  cases  which  have 
recovered,  in  which  the  exact  seat  of  the  inflammation  was  uncertain, 
may  have  been  instances  of  this  form,  and  that  the  fatality  of  the 
disease  may  not  be  so  great  as  published  facts  suggest.  But  on  this 
point  only  future  observations  can  throw  light.  Chronic  external 
meningitis  which  results  from  caries  is  only  serious  in  the  compres- 
sion it  exerts  on  the  cord. 


INTERNAL   MENINGITIS.  313 

Teeatment.— The  local  form  alone  affords  an  opportunity  for 
effective  treatment.  The  first  and  most  important  element  is  the 
treatment  of  the  original  cause  of  the  disease,  the  caries,  &c.,  to  which 
the  meningitis  is  secondary.  Any  accessible  collection  of  pus  should 
be  opened,  and  if  acute  local  symptoms,  in  a  case  of  bone  disease, 
suggest  the  passage  of  pus  into  the  vertebral  canal,  the  propriety  of 
immediate  trephining  should  be  considered.  Rest,  counter-imtatiou 
to  the  spine,  especially  the  actual  cautery,  sedatives  to  relieve  the 
pain,  and  tonics  are  the  most  important  measures  in  chronic  cases. 
So  far  as  the  acute  form  is  open  to  treatment,  the  measures  suital)le 
are  those  for  the  internal  variety.  In  a  subacute  case,  a  free  exit 
should  be  afforded  to  the  pus.  An  opportunity  for  this  very  seldom 
presents  itself,  but  may  perhaps  be  furnished  by  sufficiently  frequent 
examination  more  often  than  has  hitherto  been  the  case. 


INTERNAL    MENTNGITIS. 


Internal  meningitis,  inflammation  beginning  within  the  dura-matral 
sheath,  may  be  either  acute  or  chronic.  The  acute  form  usually  com- 
mences in  the  pia  mater  and  arachnoid ;  the  chronic  forms  may  begin 
in  these  membranes,  or  in  the  inner  surface  of  the  dura  mater.  Little 
is  known  practically  of  any  separate  affection  of  the  arachnoid. 

Acute  Internal  Meningitis. 

Internal  meningitis,  for  the  reason  already  mentioned,  has  been 
termed  "leptomeningitis,"  but  the  acute  form  only  remains  limited 
to  those  membranes  when  of  slight  degree.  In  some  cases  the  inner 
surface  of  the  dura  mater  is  also  inflamed.  Frequently  the  inflam- 
mation extends  rapidly  to  the  cord,  and  to  such  cases  the  term 
"  meningo-myelitis  "  is  often  applied ;  in  other  cases,  strange  to  say, 
no  extension  to  the  cord  takes  place.  The  inflammation  may  be 
either  simple  or  purulent,  or  it  may  be  secondary  to  tubercle.  It 
also  occurs  in  conjunction  with  cerebral  meningitis  in  the  epidemic 
form.     Acute  simple  spinal  meningitis  is  a  rare  disease. 

The  immediate  causes  that  have  been  traced  are  in  part  such  as 
have  a  local  action ;  in  part  they  are  general  morbid  influences,  such 
as  give  rise  to  other  internal  inflammations,  and  have  been  effective 
in  causing  meningitis  in  consequence  either  of  some  personal  predis- 
position, or  in  consequence  of  a  peculiarity  in  the  agent,  which  led  to 
a  specific  action  on  these  structures. 

(a)  The  local  causes  may  be— (1)  Injuries  to  the  spine,  severe  or 
slight,  from  fracture  to  simple  dislocation ;  concussion ;  surgical 
procedures,  such   as   an  operation  on   the  vertebral   column,  or  the 


314  SPINAL    MEMBRANES. 

puncture  of  tlie  sac  in  spina  bifida.  (2)  The  exposure  of  the  back  to 
cold  has  been  the  apparent  cause  in  rare  cases ;  and  still  more  rarely 
the  prolonged  exposure  of  the  spine  to  the  sun,  "  spinal  insolation," 
has  seemed  to  be  effective.  (3)  Adjacent  inflammation  has  sometimes 
caused  internal  meningitis,  but  in  such  cases  external  meningitis  has 
of  necessity  been  first  produced,  and  the  inflammation  has  passed 
through  the  dura  mater.  Hence  all  the  causes  of  acute  external 
meningitis  are  also  occasional  causes  of  the  internal  form.  But,  as  al- 
ready stated,  such  extension  through  the  sheath  is  very  rare,  and  there 
is  some  doubt  as  to  the  precise  nature  of  many  of  the  cases  in  which  it 
has  been  supposed  to  occur.  "We  have  seen  that  in  many  of  the 
instances  in  vfhich  external  meningitis  has  been  ascribed  to  adjacent 
suppuration,  the  latter  has  been  secondary,  and  the  meningitis  has 
been  the  result  of  a  primary  blood-state.  When  in  such  cases  there 
has  been  both  internal  and  external  meningitis,  it  is  probable  that  the 
former  has  not  been  due  to  actual  extension  through  the  dura  mater, 
but  that  both  have  been  the  simultaneous  result  of  a  general  cause. 

(b)  In  a  few  cases  acute  internal  meningitis  is  due  to  the  extension 
of  inflammation  from  the  cerebral  membranes.  Such  extension  is 
rarely  traced,  however,  except  in  two  classes  of  cases  : — (1)  Those  in 
which  slight  meningitis  is  found  in  the  upper  cervical  region,  as  far 
as  gravitation  favours  the  descent  of  solid  particles  in  the  cerebro- 
spinal fluid  as  the  patient  lies  in  bed.  The  inflammation  ceases  when 
the  spine  becomes  horizontal :  for  the  most  part  symptoms  are  slight 
or  absent  in  such  cases.  (2)  In  cases  in  which  a  general  spinal 
meningitis  has  been  secondary  in  point  of  time  to  inflammation  of  the 
cerebral  membranes  the  affection  has  generally  been  due  to  some 
cause  capable  of  acting  upon  both,  such  as  the  local  presence  of 
tubercles  or  a  morbid  blood-state  of  the  character  to  be  immediately 
mentioned. 

(c)  The  most  acute  and  severe  forms  of  internal  meningitis  are 
due  to  a  morbid  blood-state,  either  septicaemia  or  a  virus  allied  to  that 
which  causes  the  cerebro-spinal  form.  Purulent  meningitis  occurs  in 
cases  of  surgical  and  puerperal  septicaemia,  both  with  smd  apart  from 
a  similar  affection  of  the  cerebral  membranes.  Acute  inflammation, 
purulent  when  intense  and  prolonged,  occurs  also  as  an  isolated 
malady,  sometimes  without  apparent  exciting  cause,  sometimes  after 
exposure  to  cold.  In  such  cases  organisms  have  been  found  in  the 
spinal  fluids,  analogous  to  those  of  the  cerebro-spinal  form,  in  connec- 
tion with  which  the  pathological  relations  will  be  more  fully  con- 
sidered. It  probably  results  from  a  blood- state  not  far  removed 
from  those  which  cause  some  other  forms  of  internal  inflammation, 
and  which  ^ive  rise  to  acute  articular  rheumatism.  General  exposure 
to  cold  must  be  assumed  to  cause  so  special  an  effect  only  through 
the  agency  of  toxaemia,  such  as  that  which  has  been  already  con- 
sidered as  underlying  the  rheumatic  form  of  acute  polyneuritis.  Such 
exposure  has  been  apparently  rendered  effective  in  some  instances  by 


INTERNAL    MENINGITIS.  315 

coincident  menstruation,  wliich  may  have  produced  a  special  suscepti- 
bility to  the  attack,  or  favourable  conditions  for  the  development  of 
some  organised  virus,  such  as  seems  to  be  the  essential  factor  in  the 
causation  of  these  forms.  The  exposure  to  cold  that  has  been  effective 
has  presented  all  the  variations  that  are  met  with  in  the  case  of 
other  diseases  thus  produced,  variations  which  are  common  to  so 
many  maladies  thab  they  can  have  no  special  relation  to  any  one  of 
them.  Lastly,  as  a  crucial  example  of  this  origin  of  the  disease,  is 
the  important  fact  that  it  may  follow,  or  occur  in  the  course  of,  certain 
acute  specific  diseases,  as  one  consequence  of  the  virus  that  produces 
them. 

Pathological  Anatomy. — Internal  meningitis  is  usually  of  wide 
extent,  since  the  inflammation  spreads  readily  in  the  loose  tissue  of 
the  arachnoid.  Probably  also  the  movement  of  the  cerebro-spinal 
fluid  aids  in  the  extension  of  local  forms  and  in  the  descent  of  intra- 
cranial inflammation  by  conveying  irritant  material.  In  the  case  of  a 
patient  who  had  been  in  bed  for  some  time  the  precise  level  at  which 
such  slight  descending  meningitis  ceases  may  be  that  at  which  the 
vertebral  column  becomes  horizontal.  In  the  earliest  stage  the  only 
change  is  congestion  in  the  pia  mater,  which  is  reddened  from  vascu- 
larity, and  may  be  dotted  with  ecchymoses.  The  inner  surface  of  the 
dura  mater,  and  the  substance  of  the  spinal  cord,  may  be  similarly  con- 
gested. When  the  inflammation  is  further  advanced,  in  the  stage  in 
which  the  condition  most  often  comes  under  observation,  the  pia 
mater  and  arachnoid  are  opaque  and  thickened,  and  an  "  exudation  '* 
of  inflammatory  products,  greyish  yellow  in  tint,  may  cover  the 
pia  mater  and  occupy  the  meshes  of  the  arachnoid,  forming  a  layer 
over  the  cord.  This  exudation  varies  in  its  consistence,  and  may 
be  semi-purulent  in  aspect ;  in  the  suppurative  form  the  membranes 
are  infiltrated  with  pus,  which  also  covers  their  surface.  The  inner 
surface  of  the  dura  mater  usually  presents  similar  changes,  and 
the  inflammatory  products  may  fill  up  the  whole  space  between 
the  dura  and  pia  mater,  thus  connecting  the  two  membranes  and 
surrounding  the  nerve-roots  (Fig.  94).  The  microscope  shows 
abundant  leucocytal  elements,  and  the  larger  round  and  spindle  cells 
that  are  common  in  all  inflammatory  products.  The  former  corpuscles, 
which  resemble,  and  are  probably  identical  with  pus-corpuscles,  may 
be  abundant,  even  when  the  exudation  has  not  a  distinctly  purulent 
aspect ;  when  it  has,  they  constitute  almost  the  Avhole  of  the  material, 
lying  among  the  fibres  of  the  arachnoid  (Pig.  95).  The  vessels  are 
dilated,  and  tlieir  sheaths  distended  with  cells.  The  spinal  fluid  is 
increased  in  quantity,  and  turbid  from  flocculi,  or  even  purulent  in 
aspect.  The  nerve-roots  are  covered  with  exudation,  and  are  often 
swollen  and  reddened  from  invasion  by  the  inflammation.  But  they 
do  not  always  suffer,  even  in  purulent  meningitis,  as  Pig.  95  shows,  in 
which  a  nerve-root  is  almost  normal  in  aspect,  and  its  sheath  is 
unaffected,  altbough  it  is  surrounded  by  pus-ceHa.     The  spinal  cord 


316 


SPINAL   MEMBKANES. 


is  often  invaded  by  the  inflammation ;  it  is  then  reddened,  pale,  and 
softened,  and  the  microscope  shows  the  tissue  changes  common  in 


F.G.  94. 


/   V'' 


KjC}^ 


y^<j 


Fia.  94. — Purulent  meningitis  j  portion  of  spinal  cord  and  membranes  ;  the  space 
between  the  pia  mater  and  dura  mater  is  occupied  by  inflammatory  products,  pus, 
&c.,  in  which  the  nerve^roots,  n  n,  are  embedded.  a  a.  Cavities  which  had 
apparently  been  filled  with  liquid  pus.  Prom  a  case  of  septic  origin,  secondary  to 
caries  of  the  jaw. 

Fig-.  95. — From  the  same;  meshes  of  arachnoid  infiltrated  with  pus-corpuscles. 
A  nerve-root,  «.r.,  although  surrounded  by  pus, is  perfeotly  normal;  and  so  also  are 
the  pia  mater, ^.w.,  and  the  peripheral  layer  of  the  spinal  cord,  s.e, 

other  forms  of  myelitis.  The  change  is  always  most  marked  in  the 
periphery  of  the  cord,  and  may  occur  in  wedge-shaped  areas,  having 
the  apex  directed  inwards,  and  coalescing  at  the  surface.  The  peri- 
pheral arterioles  come  from  the  pia  mater  (see  Vessels),  and  the 
continuation  of  their  perivascular  spaces  with  the  lymphatic  interstices 
of  the  membranes  renders  it  easy  to  understand  the  invasion  of  the 
oord.  Nevertheless  in  some  cases,  especially  of  purulent  meningitis, 
the  pia  mater  itself  is  little  affected,  even  when  the  arachnoid  is  filled 
with  pus,  and  in  these  cases  the  spinal  cord  may  be  normal,  as  in  the 
example  shown  in  Fig.  94.  If  recovery  takes  place,  the  inflammatory 
products  may  undergo  cicatricial  changes,  the  membranes  remaining 
opaque  and  adherent,  and  there  may  be  a  permanent  excess  of  arach- 
noid fluid.  The  changes  in  the  cord  may  lead  to  sclerosis,  widely 
spread,  or  limited  to  certain  spots,  and  from  these  secondary  degene- 
rations, ascending  and  descending,  may  develop. 

The  area  affected  varies  in  different  cases;  the  membranes  are 
involved  in  their  whole  extent  in  most  acute  forms.  Usually  the 
affection  is  greater  on  the  posterior  than  on  the  anterior  surface ; 
probably  on  account  of  the  influence  of  the  recumbent  posture,  which 
determines  the  passage  backwards  of  the  lymphatic  fluids  containing 
material  caDable  of  exciting  and  increasing  the  inflammation. 


INTERNAL  MENINGITIS.  317 

In  tubercular  inflammation  tlie  amount  of  exudation  is  usually 
small,  and  it  may  be  absent.  It  is  often  grey  and  gelatinous  id 
appearance,  and  in  it  are  scattered  the  greyisb  or  wliitish  tubercular 
granulations.  Similar  granulations  may  usually  be  found  in  abundance 
upon  the  inner  surface  of  the  dura  mater.  Such  grey  granulations 
are  often  found  upon  the  spinal  membranes  in  cases  of  tuberculosis 
when  there  is  no  inflammation,  even  when  the  cerebral  membranes 
are  intensely  inflamed.  The  arachnoid,  especially  that  covering  the 
Cauda  equina,  may  appear  as  if  dusted  over  with  grey  particles,  so 
abundant  are  the  granulations. 

In  many  cases  the  signs  of  spinal  meningitis  are  associated  with 
those  of  inflammation  of  the  membranes  of  the  brain,  especially  of 
those  about  the  base  and  posterior  part  of  the  brain.  The  continuity 
of  the  spinal  and  cerebral  inflammation  may  be  obvious  or  indistinct. 
In  the  latter  case  the  connecting  inflammation  has  apparently  been 
much  slighter  than  that  in  the  base  of  the  brain  or  around  the  cord, 
so  slight,  indeed,  as  to  have  left  no  distinct  traces,  the  inflamma- 
tion having  apparently  extended  in  consequence  of  the  passage  of 
morbid  material  by  the  cerebro-spinal  fluid.  In  cases  of  slight  cere- 
bral meningitis,  in  which  the  patient  has  been  recumbent,  signs  of 
spinal  meningitis,  opacity  of  the  arachnoid  and  its  distension  with 
slightly  turbid  fluid,  may  exist  only  in  the  cervical  region,  ceasiag 
opposite  the  upper  dorsal  vertebrse,  and  thus  extending  as  far  as 
gravitation  (the  head  being  slightly  raised  on  a  pillow)  favoured 
the  descent  of  inflammatory  products.  But  in  the  violent  purulent 
cerebro-spinal  meningitis,  which  is  occasionally  produced  by  a  septic 
influence,  pus  around  the  cord  is  often  continuous  with  that  which 
bathes  the  base  of  the  brain.  In  a  case  of  this  kind,  in  which  the 
meningitis  was  secondary  to  acute  double  purulent  otitis,  Leyden 
found  abundant  active  micrococci,  very  similar  to  those  associated 
with  erysipelas.  In  other  acute  cases  organisms  have  been  found 
similar  to  those  of  pneumonia,  and  met  with  also  in  the  epidemic 
cerebro-spinal  form. 

Symptoms. — Slight  pain  in  the  back,  and  malaise,  may  precede  the 
acute  onset  of  the  symptoms.  This  is  usually  marked  by  a  rigor,  by 
pyrexia,  and  by  severe  pain  in  the  back.  The  latter  varies  in  posi- 
tion according  to  the  locality  of  the  inflammation,  but  is  often  felt 
along  the  whole  extent  of  the  spine.  Pain  also  radiates  along  the 
distribution  of  the  nerves,  round  the  trunk,  or  to  the  extremities. 
This  excentric  pain  is  paroxysmal  and  intense ;  sharp,  darting,  burn- 
ing, or  constricting.  The  pain  in  the  back  is  usually  constant,  with 
exacerbations.  It  is  often  increased  by  movement,  usually  also  by 
pressure,  and  by  the  application  of  a  hot  sponge  to  the  skin.  It  is  no 
doubt  due  to  the  irritation  of  the  inflamed  meninges,  while  the  radi- 
ating pain  is  produced  by  the  irritation  of  the  sensory  nerve-roots. 

Muscular  spasm  usually  comes  on  at  the  same  time  as  the  pain. 
It  shows  itself  first  in  rigidity  of  the  muscles  of  the  back,  most 


S18  SPINAL  MEMBEANES. 

marked,  if  the  inflammation  is  local,  in  tlie  neighbonrLood  of  the 
mflamed  part.  This  rigidity  is  an  important  and  characteristic 
symptom.  It  is  often  first  observed  in  the  neck,  probably  on  account 
of  the  mobility  of  the  part.  When  slight  it  may  merely  cause 
retraction  of  the  head,  or  stiffness  of  the  back,  or  may  be  so  general 
and  so  severe  as  to  cause  opisthotonos,  resembling  that  of  tetanus. 
The  spasm  usually  involves  also  other  trunk  muscles,  especially 
those  of  the  abdomen,  which  become  hard  and  cramped.  The  limbs 
also  become  rigid,  and  painful  cramp-like  spasms  occur  in  them, 
especially  on  attempts  to  move.  The  spasm  is  probably  partly  due  to 
the  irritation  of  the  motor  nerve-roots,  and  is  partly  reflex,  from  the 
irritation  of  the  sensory  roots  and  the  nerves  of  the  pia  mater.  There  is 
usually  great  hyperaesthesia  of  the  skin  to  all  forms  of  stimulation,  and 
also  increased  sensitiveness  of  the  muscles,  especially  in  the  lower 
limbs.  Pressure  on  the  arms  may  cause  no  uneasiness,  while  a 
similar  pressure  on  the  legs  occasions  great  pain.  Reflex  action  is 
usually  increased  at  the  beginning.  Constipation  is  common,  and  so 
is  retention  of  urine,  in  spite  of  irritable  attempts  to  empty  the 
bladder — the  result,  apparently,  of  spasm  of  its  muscles,  including 
the  sphincter,  which  resists  the  action  of  the  detrusor.  Dyspnoea 
may  result  from  the  spasm  of  the  thoracic  muscles,  and  may  be 
almost  suffocating  in  its  severity.  The  pulse  may  be  frequent  oi 
retarded.  The  temperature  is  raised,  sometimes,  however,  to  only  a 
slight  degree.  Cerebral  symptoms,  headache,  delirium,  coma,  occur 
when  inflammation  has  extended  within  the  skull.  The  "Cheyne- 
Stokes  breathing  "  may  be  present,  from  implication  of  the  medulla. 
The  function  of  the  vaso-motor  nerves  (which  leave  the  cord  by  many 
of  the  anterior  roots)  is  also  deranged;  the  dilatation  of  the  vessels, 
that  follows  a  stroke  on  the  skin  of  the  trunk,  is  excessive  and  pro- 
longed (meningeal  streak,  tache  sjnnale). 

As  the  disease  progresses,  the  symptoms  of  irritation  give  place  to 
those  of  paralysis,  which  may  be  most  marked  where  the  rigidity  was 
greatest.  The  limbs  become  relaxed,  and  feeble  or  powerless.  Sensi- 
bility becomes  lessened  or  lost.  Eeflex  action  disappears,  the  mus- 
cular power  in  the  limbs  becomes  so  reduced  that  the  patient  is 
scarcely  able  to  move ;  the  heart  shares  the  universal  prostration,  and 
death  may  occur  from  asthenia,  or  from  paralysis  of  the  respiratory 
muscles.  Towards  the  end  there  is  sometimes  considerable  rise  of 
temperature.  In  some  cases  the  symptoms  become  less  progressive 
and  the  disease  passes  into  a  less  acute  stage ;  the  pains  persist,  the 
loss  of  power  continues  and  may  even  slowly  increase.  Death  may 
occur,  after  weeks  of  suffering,  from  the  effects  of  bedsores  or  from 
secondary  kidney  disease,  due  to  the  retention  of  urine,  and  facilitated 
by  trophic  derangement.  On  the  other  hand,  in  slight  cases,  the  signs 
of  irritation  may  lessen  and  pass  away,  while  those  of  structural 
damage,  paralysis  and  anaesthesia,  may  remain,  and  to  these  may  be 
added  muscidar  atrophy  and  contractures,  from  the  secondary  conse- 


INTERNAL    MENINGITIS,  819 

quences  of  tlie  lesions  of  tlie  nerve-roots.  Such  persistent  symptoms 
vary  mucli  in  extent  and  degree,  according  to  tlie  position  and  intensity 
of  the  morbid  process.  Ultimately  the  symptoms  of  damage  to  the 
cord  may  either  slowly  increase,  in  consequence  of  the  spread  of 
chronic  myelitis  set  up  by  the  acute  mischief,  or,  on  the  other  hand, 
the  symptoms  of  the  meningitis  may  pass  entirely  away. 

The  symptoms  above  described  vary  in  their  distribution,  according 
to  the  position  of  the  disease.  When  the  membranes  over  the  lumbar 
enlargement  are  chiefly  affected,  the  pains,  hypersesthesia,  and  cramps 
are  confined  to  the  legs  and  loins.  When  the  disease  is  in  the  dorsal 
region  there  may  be  similar  hypersesthesia  and  spasm  in  the  legs,  but 
the  pain  and  cramp  extend  higher,  and  involve  the  trunk.  If  the 
cervical  region  is  affected,  the  symptoms  extend  to  the  upper  extremi- 
ties, the  dyspnoea  may  be  great,  and  there  is  often  dif&culty  in 
swallowing.  The  action  of  the  heart  is  sometimes  deranged,  and  con- 
traction or  dilatation  of  one  or  both  pupils  may  occur.  Extension 
to  the  brain  is  marked  by  vomiting,  general  headache,  delirium,  and 
paralysis  of  cranial  nerves,  of  which  the  first  to  suffer  are  the  spinal 
accessory  and  hypoglossal.  If  such  symptoms  preceded  those  of 
spinal  meningitis,  we  may  conclude  that  the  inflammation  commenced 
within  the  skull. 

The  symptoms  vary  somewhat  according  to  the  nature  of  the  in- 
flammation. In  purulent  meningitis,  as  already  pointed  out,  the 
symptoms  of  irritation  are  sometimes  very  slight,  apparently  because 
there  is  little  tendency  to  invade  the  nerve-roots.  In  the  case  from 
which  Figs.  94  and  95  were  taken  there  were  hardly  any  symptoms  to 
suggest  meningitis,  and  although  there  was  paraplegia,  it  was  probably 
produced  by  the  mere  pressure  of  a  large  collection  of  pus  on  the 
spinal  cord;  there  were  no  pains  or  spasm.  The  case  was  one  of 
septic  origin.  It  was  apparently  a  pure  arachnitis,  and  the  tissue  of 
the  pia  mater  was  able  to  resist  invasion  so  completely  as  to  protect 
the  structures  beneath  it  even  from  disturbance  of  function. 

The  duration  of  the  acute  symptoms  varies  from  a  day  or  two,  in 
severe  cases,  which  end  in  death,  to  two  or  three  weeks,  in  cases  of  less 
severity,  which  may  end  in  either  death  or  recovery.  The  duration  of 
the  subacute  and  chronic  symptoms  that  supervene  is  to  be  measured 
by  months,  and  sometimes  by  years. 

The  symptoms  of  tubercidar  spinal  meningitis  resemble  those  which 
have  been  described,  but  are  usually  less  intense.  There  is  pain  in 
the  back  and  loins,  with  stiffness  of  the  spine  and  retraction  at  the  neck, 
so  that  it  may  be  dif&cult  to  bend  the  head  forwards.  There  are  also 
variable  rigidity,  hypersesthesia,  and  tingling  in  the  limbs,  followed  by 
lessened  sensibility  and  paraplegic  weakness.  These  symptoms  occa- 
sionally come  on  in  the  later  stage  of  cerebral  tubercular  meningitis. 

DrA.GNOSis. — The  diagnosis  of  the  disease  rests  on  the  pain  in  the 
back,  the  retraction  and  rigidity  of  the  neck  and  spine,  the  hyperses- 
thesia and  spasm  in  the  limbs,  excited  especially  by  attempts  to  move 


820  SPINAL    MEMBRANES, 

them,  on  the  acute  onset  of  the  symptoms,  and  on  their  association  with 
pyrexia.  This  grouping  of  the  symptoms  is  sufficiently  characteristic, 
as  is  shown  by  the  fact  that  it  is  extremely  rare  for  them  to  be  simulated, 
even  remotely.  The  chief  difficulty  in  actually  acute  cases  is  presented 
by  the  forms  that  run  an  almost  latent  course,  especially  by  the  secon- 
dary purulent  form,  which  has  so  little  tendency  to  invade  the  nerve 
structures,  and  a  correspondingly  slight  tendency  to  derange  their 
functions,  and  even  to  irritate  them.  In  pure  myelitis,  on  the  other 
hand,  pain  in  the  back  is  absent  or  trifling ;  paralysis  occurs  early  and 
is  the  leading  symptom,  and  there  is  little  or  no  spasm  in  the  limbs 
in  the  early  stage  of  the  affection.  Often,  however,  some  meningitis 
occurs  at  the  onset  of  acute  myelitis,  and  then  some  pain  in  the  back 
and  slight  rigidity  in  the  limbs  may  precede  or  accompany  the  onset 
of  the  paralysis.  In  such  cases  the  predominance  of  the  meningeal 
or  cord  symptoms  must  determine  the  category  in  which  the  case  is  to 
be  placed.  Meningeal  hsemorrhage  most  nearly  resembles  meningitis 
in  its  manifestations,  and  does  so  necessarily,  because  it  produces 
inflammation ;  the  distinction  chiefly  depends  on  the  onset,  and  will 
be  considered  in  the  next  section.  Hemorrhage  into  the  spinal  cord 
can  scarcely  be  confused  with  inflammation  of  the  membranes,  be- 
cause the  only  common  symptom,  pain  in  the  back,  is  confined  to  a 
definite  spot. 

Tetanus  is  attended  by  rigidity  of  the  back,  and  by  spasm,  and 
presents  a  closer  initial  resemblance  to  meningitis  than  most  other 
affections  with  which  it  may  be  confounded ;  but  there  is  no  fever 
at  the  onset,  trismus  is  an  early  and  obtrusive  symptom,  and  the 
paroxysms  of  muscular  spasm  are  excited  by  peripheral  impressions 
much  more  readily  than  in  meningitis,  in  whif;h  they  occur  chiefly  on 
attempts  to  move.  Only  the  "rheumatic"  form  of  tetanus  would 
give  rise  to  difficulty ;  the  onset  of  the  traumatic  form  after  an  injury 
should  prevent  any  doubt.  Rheumatism  of  the  muscles  may  cause 
pain  in  the  back  on  movement,  and  the  resulting  rigidity  may  prevent 
movement ;  but  there  is  not  the  spontaneous  pain  which  attends 
meningitis,  nor  is  there  distant  spasm.  Some  diffictdty  may  be 
caused  by  rheumatism  of  the  cervical  muscles  in  young  children ;  this 
may  lead  to  retraction  of  the  head,  and  if  it  comes  on  acutely,  after 
exposure  to  cold,  considerable  doubt  as  to  its  nature  may  at  first  be 
felt.  But  it  remains  stationary,  and  the  freedom  of  the  patient  from 
spontaneous  or  radiating  pains,  and  from  any  affection  of  the  nerves, 
soon  enables  a  reassuring  opinion  to  be  formed. 

The  diagnosis  of  the  form  of  meningitis  depends  on  the  recognition 
of  the  cause  of  the  inflammation.  When  spontaneous  and  acute  the 
case  is  probably  of  the  "  primary "  form,  which  depends  on  some 
toxic  agency,  probably  often  an  organised  virus,  and  is  really  a  partial 
"epidemic"  form.  If  it  occurs  under  the  conditions  that  give  rise  to 
surgical  septicaemia,  or  after  childbirth,  purulent  meningitis  is  almost 
certain,  and  the  inflammation  may  be  far  more  iatense  than  the 


INTERNAL    MENINGITIS.  321 

severity  of  the  symptoms  suggest.  The  diagnosis  of  tubercular  spinal 
meningitis  depends  on  the  combination  with  cerebral  meningitis, 
which  usually  precedes  the  spinal  symptoms,  and  on  the  gradual  and 
insidious  onset.  Indications  of  the  tubercular  or  scrofulous  diathesis 
are  commonly  present  in  the  state  of  other  organs,  or  to  be  ascertained 
from  the  family  history  of  the  patient. 

Peognosis. — The  prognosis  is  grave  in  all  cases.  It  is  worse  the 
more  severe  and  acute  the  symptoms,  the  higher  the  temperature,  and 
the  sooner  the  symptoms  of  irritation  give  place  to  those  of  paralysis. 
It  is  worse  also  when  the  disease  is  due  to  serious  lesions  of  the  spine 
or  to  tuberculosis,  than  when  due  to  cold,  and  worse  in  the  so-called 
"  spontaneous "  cases  than  in  those  which  result  from  traumatic 
causes.  Recovery  is  more  probable  in  middle  life  than  in  early  or 
advanced  age.  The  previous  health  of  the  patient  also  influences  the 
prognosis.  It  must  always  be  remembered  that  even  if  the  patient 
survives  the  period  of  acute  inflammation,  serious  permanent  damage 
may  remain. 

Treatment.— Perfect  rest  and  quiet  are  of  the  greatest  importance 
throughout  the  course  of  the  disease.  All  sounds  should  be,  as  far  as 
possible,  excluded ;  the  light  should  be  subdued,  and  all  bodily  move- 
ment and  mental  exertion  as  far  as  possible  avoided.  The  vascular 
disturbance  of  inflammation  is  intensified  by  all  functional  excite- 
ment of  the  too  irritable  structures,  and  continuous  freedom  from 
such  excitement,  as  far  as  it  is  possible  to  obtain  the  freedom,  is  of 
paramount  importance.  Every  attack  of  spasm  means  greater  vas- 
cular disturbance,  and  the  circulatory  derangement  entails  leucocytal 
infiltration  and  other  structural  consequences. 

Although  it  is  undesirable  that  the  spine  should  be  the  lowest  part 
of  the  body,  yet,  in  acute  meningitis,  it  is  scarcely  possible  for  the 
patient  to  lie  in  any  other  posture.  The  prone  position  interferes 
with  respiration,  and  both  it  and  a  lateral  posture  entail,  directly  or 
indirectly,  greater  muscular  exertion,  and  hence  more  frequent  and 
more  severe  attacks  of  spasm,  with  secondary  harm  out  of  proportion 
to  that  which  the  posture  could  prevent.  Here,  as  so  often  in  thera- 
peutics, skill  is  shown  and  success  secured,  if  it  is  within  reach,  by 
knowing  when  to  permit  no  compromise,  and  when  to  adopt  one. 
The  dorsal  position  must  be  permitted  if  in  it  the  spasm  is  much  less 
than  in  others.  Dry,  or,  in  robust  patients,  wet  cupping,  or  leeching, 
along  the  spine  may  be  employed  at  the  onset,  and  be  followed  by  the 
local  application  of  cold  in  traumatic  or  hsemorrhagic  cases,  and  of 
heat  in  others — the  principles  being  the  same  as  in  myelitis.  Counter- 
irritation,  by  blisters  or  repeated  sinapisms,  is  more  useful  when  the 
disease  is  subsiding  than  at  the  onset.  In  cases  that  are  due  to  cold, 
free  diaphoresis  often  does  good;  a  hot  air  or  vapour  bath  should 
be  employed  at  the  onset  of  the  treatment.  A  warm  bath  may  be 
followed  by  moist  packing  for  several  hours.  The  relief  thus  given 
is  sometimes  very  great.  The  bowels  should  also  be  freely  opened. 
VOL.  I.  21 


'622  SPINAL    MEMBEANES. 

The  only  internal  remedy  wticli  lias  been  held,  for  long,  in  general 
repute,  as  capable  of  influencing  the  inflammatory  process,  is  mercury. 
The  confidence  placed  in  it  of  old  is  not  altogether  unwarranted.  It 
should  be  given  until  there  is  a  slight  affection  of  the  gums  (the  only 
evidence  that  enough  is  in  the  system  to  act  on  the  tissues) ,  and  in- 
unction is  unquestionably  the  best  way  of  administration.  We  know 
that  it  must  then  enter  the  blood  before  it  can  escape  by  the  bowel ; 
we  can  regulate  its  dose  as  we  need,  for  we  estimate  it  by  the  influ-. 
ence  on  the  gums  ;  and  by  rubbing  it  in  over  the  part  affected  we  can 
combine  some  counter-irritant  influence  (as  by  an  irritating  agent 
added  to  the  ointment  used),  and  we  necessarily  secure  the  simul- 
taneous action  of  the  two  agents,  for  the  largest  quantity  of  mercury 
in  the  blood  will  coincide  with  the  most  considerable  reflex  action  on 
the  vessels.  Lastly,  the  disturbance  of  the  stomach  and  bowels  is 
certainly  less  than  when  their  mucous  membrane  is  chosen  as  the 
path  into  the  body.  The  oleate  of  mercury  may  be  rubbed  in  along 
the  spine.  Iodide  of  potassium  seems  to  have  little  influence  over 
acute  inflammation. 

It  is  necessary  to  give  sedatives  for  the  relief  of  the  pain  and 
spasm,  if  the  application  of  cold  does  not  sufiice.  Of  these,  morphia, 
given  by  the  skin  or  the  mouth,  is  the  most  effective,  but  vomiting 
from  its  use  is  a  grave  drawback ;  fortunately  it  does  not  readily  occur 
under  the  circumstances.  Sometimes  inhalations  of  chloroform  are 
necessary  to  relieve  the  suffering,  and  may  be  even  m.ore  effective  than 
morphia.  In  slight  cases  relief  may  be  afforded  by  belladonna,  or  by 
atropia  injected  beneath  the  skin.  Chloral,  or  chloral  and  bromide, 
or  any  of  the  milder  sedatives,  may  be  given  if  there  is  insomnia. 
Bromide  alone,  unfortunately,  has  very  little  power  to  lessen  the  spinal 
reflex  action.  Frequently,  the  spinal  ice-bag  is  the  most  effective  and 
least  injurious. 

When  the  disease  has  passed  into  a  chronic  stage,  iodides  have 
been  thought  useful.  Counter-irritation  may  be  employed  freely 
with  advantage.  Tonics,  iron,  quinine,  and  even  strychnia  are 
beneficial.  Warm  baths,  as  those  of  Bath  and  Aix-les-Bains,  seem 
sometimes  of  service,  especially  employed  as  hot  douches  to  the 
spine.  But  the  acute  form  is  followed  by  a  tendency  to  the  slow 
subsidence  of  its  effects,  which  makes  the  influence  of  such  measures 
difficult  to  estimate.  The  local  consequences,  muscular  atrophy,  con- 
tiactiire,  &c.,  need  special  local  treatment  by  electricity,  rubbing,  and 
the  like. 

Epidemic  cerebro-spinal  meningitis  is  described  in  the  section  on 
diseases  of  the  cerebral  membranes  (Vol.  II). 

ChEONIC   iNTEENAIi   MENINaiTIS. 

Chronic  inflammation  of  the  membranes  of  the  cord,  within  the 
■dura-matral  sheath,  is  divided  into  two  forms,  according  as  it  begins 


INTERNAL    MENINGITIS.  S23 

in,  and  cTiiefly  affects,  the  dura  mater,  chronic  internal  pachy- 
meningitis, or  the  pia  mater  and  arachnoid,  chronic  leptomeningitis. 
Although  these  forms  sometimes  present  distinct  clinical  and  patho- 
logical features,  they  have,  when  of  considerable  degree,  many 
characters  in  common;  they  own  the  same  causes,  and  need  the 
same  treatment.  Hence  it  is  most  convenient  to  describe  them 
together  as  forms  of  internal  meningitis.  The  condition  termed 
hsematoma  of  the  dura  mater  depends  on  a  form  of  haemorrhagic 
inflammation. 

Chronic  meningitis  is  seldom  fatal,  and  is  rare  as  a  general  and 
primary  malady.  As  such,  therefore,  our  knowledge  of  it  is  still 
scanty,  and  the  opinions  formerly  current  have  had  to  be  largely 
curtailed.  It  was  inferred  that  chronic  symptoms,  analogous  to  those 
which,  when  acute,  are  due  to  acute  meningitis,  were  evidence  of  a 
chronic  form.  This  was,  therefore,  assumed  to  be  the  cause  of  a 
group  of  symptoms,  of  which  spasm  is  the  prominent  and  dominant 
feature,  which  are  now  known,  to  be  due  solely  to  a  morbid  state  of 
the  spinal  cord  itself,  and  to  be  consistent  with  a  perfectly  normal 
state  of  the  membranes.  Hence  "  chronic  meningitis,"  as  it  was 
recognised  twenty  years  ago,  as  a  "clinical  entity,"  has  ceased  to 
exist,  or  rather  has  passed  into  the  pathological  conception  of 
**  primary  lateral  sclerosis."  The  definite  knowledge  we  now  have  of 
general  primary  chronic  meningitis  is  limited.  The  important  fact 
regarding  it  is  that  its  symptoms  differ  from  those  of  the  acute  form 
more  widely,  perhaps,  than  do  those  of  any  other  chronic  inflamma- 
tion. Yet  this  difference  depends  on  a  comparatively  small  element — 
on  the  fact  that  only  acute  inflammation  causes  acute  irritation  of  the 
motor  and  reflex  structures.  The  chronic  form  may  irritate  the 
sensory  nerve-roots,  but  its  motor  manifestations  are  chiefly  the  result 
of  pressure,  and  even  to  this  much  of  the  pain  may  be  due.  The 
pressure  may  cause  spasm,  but  it  is  a  slow  tonic  contraction,  wholly 
unlike  that  of  the  acute  form.  The  difference  in  the  effect  of  the  two 
forms  of  inflammation  suggests  many  problems  that  deserve  investiga- 
tion, but  their  consideration  would  be  out  of  place  here. 

Causes. — Chronic  internal  meningitis,  in  every  form,  is  most 
frequent  in  adult  age,  and,  like  acute  inflammation,  affects  men  more 
frequently  than  women.  In  its  general  and  primary  form,  it  has  been 
thought  to  occur  more  readily  in  persons  with  neurotic  heredity, 
although  the  influence  of  this  is  doubtful.  Debilitating  influences  of 
various  kiads  predispose  to  the  disease,  and  prolonged  over-exertion 
has  been  thought  sometimes  to  produce  it.  .Among  exciting  causes 
the  most  important  is  severe  and  repeated  exposure  to  cold.  Trau- 
matic lesions,  concussion,  &c.,  are  occasional  causes.  It  may  result 
by  extension  from  inflammation  outside  the  dura  mater  and  from 
chronic  inflammation  of  the  substance  of  the  cord  itself.  But  their 
influence  in  producing  chronic  internal  inflammation  (except  as  a 
sequel  to  the  acute  form)  is  a  subject  on  which  the  opinions  of  the 


324  SPINAL  MEMBRANES. 

past  have  still  to  be  subjected  to  careful  comparison  with  ascertained 
facts.  It  is  probable,  however,  that  the  condition  does  sometimes 
follow  concussion  of  the  spine  that  has  no  immediate  or  acute  con- 
sequences, and  the  chronic  stage  into  which  an  acute  traumatic 
meningitis  subsides  may  last  so  long  as  to  throw  its  initial  form  into 
the  shade,  and  may  not  only  persist  with  independent  pertinacity,  but 
may  extend  without  relation  to  the  original  seat.  Thus  we  must 
distinguish  as  effects  of  injury,  the  local  and  the  general  forms.  The 
latter  are  predisposed  to  by  the  influences  that  seem  to  facilitate  the 
occurrence  of  the  primary  form,  and  in  these  cases  the  influence  of  the 
injury  often  seems  to  be  trifling,  and  the  existence  of  the  morbid  state 
is  not  always  beyond  doubt. 

Local  chronic  meningitis  may  also  result  from  any  chronic  disease 
either  ol  the  membranes  themselves,  the  bones,  or  the  spinal  cord. 
These  need  not  be  enumerated  in  detail.  It  occurs  in  aU  cases  of 
compression,  and  especially  in  all  forms  of  chronic  myelitis  that 
irsvolve  the  superficial  layers  of  the  cord. 

Lastly,  certain  general  morbid  states  are  frequent  causes.  The  first 
is-  alcoholism,  which  may  give  rise  to  general  inflammation,  involving 
both  the  pia  arachnoid  and  the  superficial  layers  of  the  cord  in  various 
degrees.  A  second  and  also  common  cause  is  syphilis,  which  may 
produce  either  pachymeningitis,  surrounding  the  cord  in  the  way 
presently  to  be  described,  or  local  leptomeningitis,  irregular  in  position 
and.  in  effects.  Lastly,  in  very  rare  cases  tubercle  has  given  rise  to  a 
chronic  inflammation,  chiefly  of  the  inner  surface  of  the  dura  mater. 
Hsemorrhagic  pachymeningitis  occurs  especially  in  the  insane,  but  has 
been  met  with  as  a  consequence  of  chronic  alcoholism  and  after 
injuries. 

Pathological  Anatomt. — In  slight  and  moderate  degree,  there  is 
merely  opacity  and  thickening  of  the  membranes  affected,  sometimes 
with  distension  of  vessels  or  minute  spots  of  extravasation.  The 
opacity  of  the  arachnoid  may  be  such  that  the  spinal  cord  cannot  be 
seen  through  it.  The  inner  surface  of  the  dura  mater  may  be 
granular  when  it  is  not  otherwise  changed.  The  spinal  fluid  is 
increased  in  quantity  and  is  turbid.  When  the  changes  are  greater 
in  degree,  the  dura  mater  and  pia  mater  may  be  connected  together 
by  a  layer  of  inflammatory  tissue  of  considerable  thickness,  so  that  it 
may  be  impossible  to  say  in  which  membrane  the  disease  commenced. 
The  microscope  shows  the  ordinary  elements  which  result  from 
inflammation,  cells  of  various  kinds,  many  lymphoid  and  pus-like 
corpuscles,  and  distended  vessels,  often  incrusted  by  similar  cells. 
Frequently  also  the  pia  mater  is  transformed  into  a  thick  irregular 
layer  of  homogeneous  tissue  in  which  no  distinct  cell-elements  can  be 
perceived,  and  only  faint  indications  of  a  fibrous  structure  (Fig.  96). 
The  walls  of  its  vessels  may  be  greatly  thickened  by  similar  material. 

The  nerve-roots  passing  through  the  diseased  membranes  very 
seldom  escape,  as  they  may  in  the  acute  form,  but  are  inflamed, 


INTBUNAL    MENINGITIS.  825 

reddened,  and  swollen  in  the  active  stage,  and  afterwards  compressed 
and  atrophied,  if  the  amount  of  new  tissue  formed  about  them  is  con- 


Fig.  96. — Chronic  alcoholic  meningitis.  Section  of  edge  of  anterior  column  :ind  of 
a  large  nerve-root ;  carmine  prepjiration.  p.m.,  pia  muter  irregularly  thickened 
and  transformed  into  amorphous-looking  tissue,  from  wliich  wedge-shaped 
branching  tracts  {x  x)  extend  into  the  white  substance  ;f.f-t  fasciculi  of  nerve- 
fibres  entering  the  cord;  a.,  an  artery  in  the  nerve-root,  enlarged  and  with 
thickened  walls;  t.t.,  tracts  of  amorphous  conneciive  tissue;  e.,  a  small 
extravasation. 

siderable.  The  fibres  suffer  especially  when  the  inflammation  in- 
volves the  dura  mater,  on  account  of  the  unyielding  character  of  the 
fibrous  sheaths  which  the  nerves  receive  from  that  membrane.  When 
the  pia  mater  is  thickened,  scattered  tracts  of  connective  tissue  may 
be  seen  in  the  substance  of  the  nerve-roots,  between  the  fibres 
(Fig.  96,  t). 

The  spinal  cord  presents  the  same  variations  in  the  degree  in  which 
it  suffers.  It  may  be  little  affected,  but  is  frequently  damaged  by  the 
extension  to  it  of  the  inflammation  of  the  pia  mater,  which  causes 
softening,  vascularity,  loss  of  distinction  between  grey  and  white 
substance,  breaking  down  of  nerve-elements,  and  infiltration  by 
lymphoid  and  other  inflammatory  cells.  Ultimately  indurating  tissue 
remains,  constituting  an  irregular  zone  of  peripheral  sclerosis.  From 
the  thickened  pia  mater  tracts  of  similar  tissue  may  extend  into  the 
cord,  becoming  narrower  as  they  pass  inwards  and  sending  out 
branching  trabeculse  (Fig.  96,  z).  Between  these  tracts  the  nerve- 
fibres  are  more  or  less  damaged,  partly  by  the  inflanimation,  and 
partly  by  the  compression  produced  by  the  newly-formed  tissue. 

This  peripheral  sclerosis  associated  with  chronic  meningitis,  espe- 
cially in  the  neighbourhood  of  injuries,  frequently  damages  tracts  that 
undergo  secondary  ascending  degeneration — ^postero-median  column, 


326  SPINAL   MEMBRANES. 

direct  cerebellar  tract,  and  ascending  antero-lateral  tract.  Tlie  result 
is  to  produce  a  mixed  lesion,  the  precise  nature  of  whicli  is  not  always 
easily  distinguished  and  has  certainly  often  been  mistaken.  The 
equivocal  aspect  of  the  process  is  probably  increased  hj  the  fact  that, 
as  we  have  seen,  secondary  degeneration  entails  an  interstitial  process 
that  is  prone  to  assume  an  inflammatory  character,  especially  when 
set  up  by  an  irritative  lesion.  Hence  the  peripheral  sclerosis  set  up 
by  the  meningitis  is  apt  to  carry  with  it  a  certain  amount  of  inflam- 
mation in  the  pia  mater,  far  beyond  the  region  to  which  this  would 
otherwise  be  limited.  A  similar  thickening  of  the  pia  mater  is, 
however,  met  with  over  areas  of  the  cord  that  are  the  seat  of  other 
forms  of  sclerosis,  both  those  that  are  systemic,  as  in  tabes,  and  those 
that  are  random,  as  in  the  insular  form.  It  is  probably  secondary  in  all 
cases,  but  it  formerly  gave  rise  to  some  erroneous  conceptions  of  the 
nature  of  such  degenerations  (see  Locomotor  Ataxy).  A  still  more 
considerable  implication  of  the  pia  mater  is  met  with  over  regions  that 
are  the  seat  of  focal  chronic  myelitis. 

In  the  ordinary  form  of  chronic  meningitis,  the  membrane  in  which 
the  inflammation  begins  may  be  much  more  affected  than  the  other, 
although  the  latter  is  very  rarely  healthy,  except  in  the  slightest  cases. 
Inflammation  beginning  in  the  pia  mater  and  arachnoid  is  often 
extensive  in  range,  and  may  be  considerable  or  sHght  in  degree.  That 
which  commences  on  the  inner  surface  of  the  dura  mater  is  frequently 
limited  in  extent,  but  is  accompanied  by  the  formation  of  a  large 
amount  of  new  tissue,  and  hence  has  been  termed  hypertrophic  internal 
pachymeningitis.  It  constitutes  a  very  important  variety,  giving 
rise  to  peculiar  and  grave  symptoms  which  were  first  carefully  studied 
by  Charcot  and  Joffroy.  It  affects  most  commonly  the  cervical  region, 
but  sometimes  occurs  at  the  lower  part  of  the  cord.  On  opening  the 
spinal  canal  a  fusiform  tumour  is  seen,  the  outer  surface  of  which  is 
the  unaltered  outer  surface  of  the  dura  mater,  and  on  section  the 
enlargement  is  found  to  depend  chiefly  on  a  great  thickening  of  the 
inner  part  of  this  membrane,  sometimes  amounting  to  a  quarter  of  an 
inch ;  several  layers  of  new  tissue  can  often  be  distinguished.  The 
pia  mater  may  be  normal,  but  is  more  commonly  thickened,  and  it  is 
often  united  by  the  thickened  arachnoid  to  the  tissue  proceeding  from 
the  dura  mater.  The  cord  is  compressed  and  commonly  softened  at 
the  spot,  and  it  presents  signs  of  inflammation.  The  nerve-roots  are 
also  damaged  and  compressed  by  the  newly -formed  tissue.  Some- 
times at  the  spot  most  affected,  the  cord  may  be  surrounded  by  a  ring 
of  new  tissue  of  cartilaginous  hardness.  In  other  cases  the  thickening 
of  the  dura  mater  is  slighter  and  more  diffuse,  affecting  occasionally 
a  wide  extent  of  the  membrane.  In  rare  cases  the  membranes  about 
the  Cauda  equina  may  alone  be  affected — the  dura  and  pia  mater 
adherent,  and  the  nerves  united  in  a  fibrous  mass. 

The  white  fibroid  or  cartilaginous  plates  found  so  often  in  the 
arachnoid    after   death  are  probably  not  connected  with  preceding 


INTERNAL  MENINGITIS.  827 

inflammation.  In  most  cases  in  which  they  are  found,  no  symptoms 
haTe  existed  during  life.  It  is  said,  however,  that  when  they  are 
numerous  and  extensive  they  may  give  rise  to  symptoms  closely 
resembling  those  of  chronic  meningitis  (Yulpian).  But  fibroid  plates 
in  the  dura  mater  have  been  seen  in  a  case  in  which  previous 
symptoms  suggested  that  they  resulted  from  chronic  inflammation 
(Jaccoud). 

The  rare  condition  termed  internal  hsemorrhagic  pachymeningitis,  or 
hsematoma  of  the  spinal  dura  mater,  is  similar  to,  and  commonly 
associated  with,  that  which  affects  the  cranial  membrane,  and  will 
be  afterwards  described.  In  this,  a  reddish-brown  exudation  covers 
the  surface  of  the  membrane,  and  is  composed  of  fibrin  and  extrava- 
sated  blood ;  the  latter  may  be  encysted  in  small  cavities,  or  may  be 
in  various  stages  of  transformation.  The  change  commonly  extends 
over  a  great  part  of  the  dura  mater,  and  is  apparently  the  result  of 
haemorrhage  into  inflammatory  tissue. 

The  local  form  of  syphilitic  meningitis  presents  an  irregular  thick- 
ening of  the  pia  mater  and  arachnoid,  with  the  extensive  pi'oduction 
of  new  tissue  in  the  affected  region  characteristic  of  this  variety,  so 
that  it  often  resembles  a  diffuse  growth  as  much  as  a  chronic  inflam- 
mation. The  new  tissue  has  a  peculiar  gluey  aspect,  and  presents  a 
tendency  to  caseous  degeneration  and  fibroid  change,  so  that  scattered 
or  coalescing  cheesy  spots  are  ultimately  met  with  in  tracts  of  mixed 
fibrous  and  gelatinous  aspect,  the  latter  appearance  predominating  in  the 
more  recent  parts.  This  process  of  growth  may  attain  such  a  develop- 
ment at  some  spot  as  to  constitute  a  distinct  "  gumma,"  which  may 
invade  the  substance  of  the  cord,  growing  inwards  from  the  pia  mater. 
A  similar  process  of  cell- growth  may  be  sometimes  traced  with  the 
microscope  into  the  substance  of  the  cord,  along  the  tracts  of  neuroglial 
tissue  and  peri-arterial  spaces,  when  there  is  no  invasion  distinct  to 
the  naked  eye.*  A  more  limited  affection  of  the  walls  of  the 
arteries  of  the  pia  mater,  extending  into  the  cord,  has  also  been 
thought  to  be  syphilitic  in  nature ;  the  whole  thickness  of  the  wall  of 
the  arterioles  may  be  the  seat  of  inflammatory  thickening,  in  conse- 
quence of  which  the  calibre  of  the  vessels  is  narrowed.f  There  is, 
however,  some  reason  to  think  that  a  similar  affection  of  the  vessels 
may  occur  apart  from  syphilis. J 

In  the  very  rare  chronic  tubercular  meningitis  the  inner  surface  of 
the  dura  mater  is  covered  with  a  layer  or  layers  of  tubercles,  in  various 
stages  of  development,  mingled  with  inflammatory  products.  The 
arachnoid  may  also  contain  degenerating  tubercles,  and  the  two 
membranes  may  be  united  together  by  the  new  formation.  The 
condition  occurs  independently  of  bone  disease.  § 

*  Gilbert  and  Lion, '  Arch,  gen.,'  1889. 

t  Schmaus,  '  Deut.  Arch.  £.  kl.  Med.,'  1889. 

X  Hochhaus,  '  Thesis,'  Kiel,  1889. 

§  Weiss, '  Wiener  med.  Wocbensch.,'  1885. 


328  SPINAL   MBMBHANES. 

Symptoms. — The  symptoms  of  clironic  internal  meningitis,  like 
those  of  the  acute  form,  are  due  to  the  irritation  of  the  membranes, 
to  the  damage  to  the  nerve-roots,  and  to  that  of  the  cord.  But  the 
absence  of  acuteness  in  the  process  involves,  as  we  have  seen,  a  dif- 
ference which  produces  a  contrast,  rather  than  a  resemblance,  in  the 
manifestations  of  the  two  diseases  ;  the  spasm  which  dominates  the 
aspect  of  acute  meningitis  is  almost  entirely  absent  in  the  chronic 
form.  When  this  is  eliminated,  similar  disturbances  of  function 
remain,  but  their  relative  subordination  renders  the  similarity  in  the 
features  of  the  two  maladies  unobtrusive.  Still,  the  three  pathological 
elements  just  mentioned  occur  in  the  chronic  as  well  as  in  the  acute 
form,  and  in  different  cases  one  or  another  of  the  corresponding  sets 
of  symptoms  predominates.  Hence  the  varieties,  while  possessing 
many  characters  in  common,  may  differ  considerably  in  their  clinical 
aspect.  In  some  cases,  we  can  infer  with  accuracy  the  special  seat 
of  the  disease,  but  in  many  others  we  cannot  carry  our  diagnosis 
beyond  the  conclusion  that  chronic  internal  meningitis  exists,  and  are 
unable  to  say,  even  with  probability,  which  membrane  is  primarily 
affected. 

The  common  symptoms  are  these :  pain  in  the  back,  increased  by 
movement, — sometimes,  however,  amounting  only  to  heavy  dull  dis- 
comfort,— and  accompanied  by  some  stiffness  of  the  bacK,  and,  when  in 
the  cervical  region,  by  some  retraction  of  the  head.  The  pain  is 
increased  by  pressure  on  the  vertebral  spine,  and  its  increase  by 
movement  may  cause  a  fixation  of  mobile  parts,  as  the  neck,  in  some 
abnormal  position.  The  pain  is  apparently  diie  to  the  ii'ritation  of 
the  meningeal  nerves,  and  the  rigidity  is  to  be  regarded  as  a  reflex 
effect  of  this  irritation.  But  these  are  not  the  paroxysms  of  spasm, 
so  conspicuous  and  so  distressing  in  the  acute  form. 

More  obtrusive,  in  most  cases,  are  the  radiating  or  excentric  pains 
due  to  the  irritation  of  the  nerve-roots,  and  referred  to  the  region  in 
which  the  nerves  are  distributed, — back  of  the  head,  neck,  arms 
thorax,  abdomen,  loins,  or  legs,  according  to  the  position  of  the  disease 
These  pains  are  often  very  severe,  sharp,  dartiwg,  burning,  or  rheu- 
matoid in  character,  paroxysmal  in  occurrence,  and  sometimes  worse 
at  night.  They  may  be  accompanied  by  a  painful  sense  of  constriction, 
and  by  various  unpleasant  sensations — numbness,  tingling,  formica- 
tion,— in  the  same  areas.  Hyperaesthesia  may  also  exist,  so  that  pain, 
sometimes  thrilling  in  character,  is  produced  by  touchi:^g  the  skin,  or 
by  sligbt  degrees  of  heat  or  cold,  and  a  hot  or  cold  sponge  passed 
along  the  skin  of  the  back  may  reveal  the  chief  seat  of  the  disease  by 
the  change  of  sensation  at  its  level,  as  in  the  acute  form.  The  hyper- 
sest  \esia  may  be  more  marked  to  one  form  of  sensation  than  to  another. 
Cutaneous  eruptions,  such  as  result  from  other  nerve  lesions,  have  also 
been  observed  in  association  with  the  pains.  In  the  same  regions, 
muscular  twitching,  tremor,  or  even  spasm,  may  resvilt  from  the  irrita- 
tion of  the  motor  lOot-fibres.     These  symptoms  correspond  in  their 


INTEENAL  MENINGITIS.  829 

level  to  tlie  part  of  the  membranes  cMefly  diseased.  When  the 
lumbar  membranes  are  affected,  the  radiating  pains  are  felt  in  the 
legs;  but  when  the  disease  is  higher  up,  the  legs  may  merely  feel 
heavy,  and  be  the  seat  of  slight  abnormal  sensations. 

After  a  time,  weeks  or  months,  the  pains  persisting  or  ceasing, 
certain  paralytic  symptoms  manifest  themselves  in  the  regions  in 
which  the  severe  pains  were  felt,  and  are  dependent  on  further  damage 
to  the  nerve-roots.  Sensation  becomes  lessened,  or  even  lost,  in 
certain  areas,  to  touch,  or  pain,  or  both.  The  muscles  in  the  same 
region  become  weak,  and  waste ;  usually  with  the  reaction  of  degene- 
ration for  a  time,  and  ultimate  loss  of  all  irritability.  The  wasting 
often  affects  the  muscles  irregularly,  from  the  unequal  damage  to  the 
roots  and  the  fibres  of  single  roots :  entire  groups  sometimes  atrophy 
from  the  complete  interruption  of  the  roots  of  one  or  more  segments. 
Eeflex  action  in  these  parts  is  lost.  In  the  trunk  this  loss  of  reflex 
action,  with  anaesthesia,  may  be  the  chief  local  symptom,  and  is  of 
m.uch  diagnostic  importance.  As  the  disease  progresses,  the  cord 
itself  suffers,  by  compression,  or  by  extension  to  it  of  the  inflamma- 
tion. The  parts  below  the  seat  of  the  disease  then  become  paralysed, 
the  legs  are  weak  and  the  seat  of  dull  heavy  pain ;  reflex  action  in 
them  may  be  increased,  while  their  muscular  nutrition  continues 
good,  unless  the  lumbar  membranes  are  diseased,  or  the  myelitis 
descends  the  cord.  Defect  of  co-ordination  in  the  legs  is  not 
uncommon,  and  is  probably  due  to  the  implication  of  the  fibres  from 
the  muscles  in  the  posterior  columns  and  cerebellar  tracts.  Power 
over  the  sphincters  may  be  lost  and  bedsores  form,  but  chiefly  in 
cases  in  which  the  disease  is  severe  over  the  lumbar  region  or  cauda 
equina. 

When  the  inflammation  affects  chiefly  the  pia  mater  and  arachnoid 
{chronic  leptomeningitis)  the  symptoms  of  meningeal  irritation  are 
conspicuous, — vertebral  pain  and  rigidity  of  the  back,  with  cutaneous 
hypersesthesia,  and  the  radiating  pains  may  also  be  considerable. 
These  symptoms  are  especially  marked  in  cases  of  subchronic  menin- 
gitis, such  as  are  sometimes  due  to  alcoholism.  The  local  paralytic 
symptoms — ansesthesia  and  muscular  wasting — maybe  absent  in  these 
cases,  or  may  only  come  on  in  slight  degree  after  the  other  symptoms 
have  lasted  for  a  considerable  time.  On  the  other  hand,  weakness 
and  pains  in  the  legs,  from  affection  of  the  cord  itself,  may  be  early 
symptoms. 

When  the  inflammation  begins  in  the  dura  mater  (internal  paclnj- 
meningitis)  the  symptoms  of  spinal  irritation,  pain  and  stiffness  of 
the  back,  although  occasionally  present,  are  much  less  prominent 
symptoms  than  are  those  which  depend  on  the  irritation  of,  and 
damage  to,  the  nerve-roots.  The  radiating  pains  are  very  severe,  and 
are  often  the  earliest  symptom.  At  a  later  stage  the  muscular  weak- 
ness, followed  or  accompanied  by  wasting,  chiefly  attracts  attention, 
and  the  wasting  may  be  so  great  that  the  disease  is  mistaken  for 


S30  SPINAL    MEMBRANES. 

progressive  miisctilar  atrophy.  This  is  especially  the  case  when  the 
lesion  is  in  the  cervical  region,  the  "cervical  hypertrophic  pachy- 
meningitis "  of  Charcot  and  Joffroy.  In  this  affection,  pains  in  the 
back  of  the  head,  neck,  shotdders,  and  arms,  often  accompanied  with 
a  painful  sense  of  constriction,  precede  the  muscular  atrophy.  The 
wasting  may  involve  many  muscles,  but  usually  those  supplied  by 
the  radial  nerve  are  less  affected  than  the  others ;  the  escape  of  the 
long  extensors  of  the  wrist  and  fingers,  while  the  flexors  of  the  wrist 
and  fingers  and  the  interossei  atrophy,  leads  to  a  peculiar  deformity ; 
the  unopposed  muscles  cause  persistent  over-extension  of  the  wrist ; 
the  phalangeal  joints  are  flexed  and  the  metacarpo-phalangeal  joints 
are  extended,  but  from  the  position  of  the  wrist  they  are  not  over- 
extended, and  the  hand  thus  differs  from  the  claw-like  hand  (p.  43). 
With  this  muscular  wasting  in  the  arms  there  is  paraplegic  weakness 
from  the  pressure  on  the  cord.  In  rare  cases  the  disease  affects  the 
membranes  over  the  lumbar  enlargement  or  Cauda  equina,  and  then 
the  legs  are  the  seat  of  the  pains,  paralysis,  and  atrophy  already 
described,  and  the  sphincters  are  early  paralysed. 

If  the  pachymeningitis  is  confined  to  the  dorsal  region,  the  root- 
symptoms  are  limited  to  the  trunk.  In  such  a  case  anaesthesia  over 
the  region  supplied  by  the  dorsal  nerves  was  associated  with  para- 
plegia from  compression  of  the  cord.  In  another,  areas  of  ansesthesia, 
with  severe  pains,  existed  over  the  legs,  trunk,  neck,  and  back  of  the 
head,  without  any  indication  of  damage  to  the  cord  itself.  These 
examples  illustrate  the  great  variations  presented  by  different  cases, 
owing  to  the  different  distribution  of  the  disease.  An  important 
characteristic  of  this  form  is  the  fact  that  it  involves  the  membranes 
equally  around  the  cord,  and  so  gives  rise  to  bilateral  symptoms. 

Chronic  syphilitic  meningitis  may  cause  symptoms  resembling 
those  of  any  other  variety  of  chronic  inflammation,  even  the  hyper- 
trophic pachymeningitis  being  sometimes  due  to  this  cause.  But 
its  most  frequent  and  special  characteristis  is  the  focal  character  of 
the  symptoms,  and  their  tendency  to  be  associated  with  those  of 
partial  and  local  damage  to  the  spinal  cord  itself.  This  is  in  con- 
sequence of  the  tendency  of  local  tissue-formation  to  be  associated 
with  the  inflammation.  Hence,  pains  of  irregular  distribution  on 
one  side  are  usually  soon  followed  by  impairment  of  sensibility,  and 
often  by  weakness  in  the  corresponding  leg. 

In  hsemorrhagic  pachymeningitis  the  symptoms  are  those  of  a 
slight  chronic  inflammation,  without  any  distinctive  features  that 
can  be  referred  to  the  hsemorrhagic  infiltration. 

Diagnosis. — The  diseases  with  which  chronic  internal  meningitis  is- 
liable  to  be  confounded,  are  different  according  as  the  symptoms  of 
irritation,  of  damage  to  the  cord,  or  of  compression  of  the  nerve-roots, 
predominate.  By  far  the  most  imjDortant  diagnostic  symptoms  are 
those  that  depend  on  the  damage  to  the  nerve-roots — their  early  irri- 
tation and  later  structural  damage. 


INTERNAL    MENINGITIS.  831 

The  spinal  pain  and  stiffness  may  be  mistalien  for  simple  racMalgia 
or  "  spinal  irritation,"  but  in  this  condition  the  "wh.ole  spine  is  tender, 
several  separate  points  are  much  more  tender  tban  the  rest,  and  there 
are  not  the  radiating  pains  or  paralytic  symptoms  of  meningitis. 
Pain  in  the  back  is  related  in  greater  degree  to  posture  and  exertion, 
and  it  has  a  tendency  to  pass  from  the  neck  to  the  occiput.  The 
patients  have  usually  suffered  from  neuralgia  in  other  situations, 
which  may  alternate  with  that  in  the  back. 

Peripheral  pains,  especially  about  the  trunk  and  legs  and  shoulders, 
are  prominent  symptoms  in  some  cases  of  muscular  rheumatism  or 
rheumatic  neuralgia.  In  these,  however,  there  are  not  the  pain  and 
tenderness  of  the  spinal  column ;  and  although  there  may  be  apparent 
increased  sensitiveness  in  the  region  of  the  pains,  it  is  of  the  deeper 
structures  and  not  of  the  skin,  while  the  relation  of  the  pain  to  move- 
ment and  muscular  action  is  a  conspicuous  feature. 

Radicular  neuritis  is  probably  associated  in  many  cases  with  some 
affection  of  the  adjacent  membranes,  but  meningeal  symptoms  are 
altogether  subordinate ;  the  symptoms  are  confined  to  the  region  sup- 
plied by  the  nerve-roots,  and  spinal  pain  is  absent. 

The  muscular  wasting  may  be  mistaken  for  atrophy  due  to  degene- 
ration or  subacute  inflammation  of  the  anterior  cornua  of  the  cord 
(progressive  muscular  atrophy,  subacute  polio-myelitis).  The  cervical 
hypertrophic  pachymeningitis  bears  special  resemblance  to  this  disease, 
since  the  wasting  in  the  arms  is  often  associated  with  paraplegic  sym- 
ptoms in  the  legs.  The  distinction  is  that  the  atrophy,  in  the 
meningeal  form,  is  preceded  by  severe  pains,  and  often  accompanied 
by  areas  of  hypersesthesia  or  anaesthesia,  and  is  random  in  distribu- 
tion. In  chronic  affections  of  the  anterior  cornua  both  pains  and 
anaesthesia  are  usually  absent,  and  the  wasting  often  affects  the 
muscles  in  a  certain  order,  which  will  be  described  in  the  account  of 
the  disease.  Thus,  in  a  man  who  was  supposed  to  have  cornual 
disease  on  account  of  wasting  of  some  miiscles  of  both  legs,  irregu- 
larly distributed,  a  history  of  severe  sharp  pains,  and  the  discovery  of 
a  patch  of  anaesthesia  on  one  leg,  the  presence  of  a  burning  pain  round 
one  side  of  the  trunk,  and  the  irregular  distribution  of  the  wasting,  led 
to  a  diagnosis  of  chronic  meningitis  damaging  the  nerve-roots ;  there 
was  a  history  of  syphihs,  and  appropriate  treatment  completely  re- 
moved the  symptoms.  The  pains  also  constitute  a  distinction  from 
another  affection  which  presents  somewhat  similar  symptoms— syringo- 
myelia. Chronic  disseminated  myelitis  may  cause  muscular  wasting, 
but  does  not  cause  the  pain  in  the  back,  or  the  rigidity  of  the  spine, 
which  occtir  in  meningitis,  and  the  paralysis  from  damage  to  the  cord 
is  the  leading  symptom.  It  must  be  remembered  that  the  two  condi- 
tions of  meningitis  and  myelitis  are  often  combined,  and  the  fact  has 
to  be  recognised  in  our  diagnosis. 

Some  authorities  believe  that  the  symptoms  of  "ataxic  paraplegia," 
which  result  from  combined  lateral  and  posterior  sclerosis,  are  often 


332  SPINAL  MEMBRANES. 

produced  by  tlie  agency  of  clironic  meniagitis,  invading  tlie  peripteral 
layers  of  the  cord.  It  is  not  probable,  however,  that  this  mecbanism 
is  operative  in  the  cases  in  wbicb  considerable  paraplegia  shows  that 
the  lateral  pyramidal  tracts  are  extensively  involved.  The  diagnostic 
problem  is  considered  in  the  chapter  on  this  disease. 

When  the  meningitis  is  lumbar,  the  pains  in  the  legs  may  resemble 
some  of  those  that  occur  in  locomotor  ataxy,  and  the  knee-jerk  may  be 
lost  in  each  disease ;  but  the  loss  in  meningitis  is  associated  with 
muscular  weakness  and  wasting,  there  is  not  the  degree  of  ataxy 
common  in  tabes,  and  true  "  bghtning  pains "  are  not  met  with  in 
meningitis.  The  greatest  resemblance  to  chronic  meningitis  is  pre- 
sented by  some  cases  of  tabes  in  which  the  cord  disease  reaches  a 
greater  degree  in  the  dorsal  than  in  the  lumbar  region.  There  are 
then  pains  and  anaesthesia  in  the  trunk,  and  the  atrophy  in  the  legs 
may  be  httle  marked.  In  meningitis,  if  the  membranes  over  the 
lumbar  enlargement  are  normal,  the  knee-jerk  is  preserved  and  often 
increased,  while  it  is  lost  in  tabes.  The  anaesthesia  and  loss  of  the 
trunk  reflexes  are  more  general  and  uniform  in  tabes  than  in  menin- 
gitis. 

Caries  of  the  spinal  bones  produces  many  of  its  symptoms  by  the 
agency  of  chronic  meningitis,  which  always  results  from  it.  Hence 
the  diagnosis  depends  on  the  recognition  of  the  presence  of  the  disease 
of  the  bone,  m  addition  to  that  of  the  membranes. 

Multiple  neuritis,  in  the  paralytic  variety,  presents  nerve- symptoms 
of  the  same  character  as  those  of  chronic  meningitis,  but  the  special 
symmetry  and  peculiar  distribution  of  the  atrophic  palsy,  the  promi- 
nence of  limb-tenderness,  and  the  absence  of  cord  symptoms,  pre- 
clude sach  similarity  as  can  cause  actual  confusion  between  the  two 
diseases. 

Peognosis. — The  differences  between  the  forms  of  chronic  menin- 
gitis, and  between  the  degrees  of  the  same  forms,  are  so  vast  as  to 
render  the  prognosis  of  the  disease  a  matter  of  individual  considera- 
tion in  every  case,  to  be  estimated  independently,  from  the  patho- 
logical characters.  The  severe  degrees  of  the  affection  are  attended 
with  danger  to  Ufe,  and  even  the  slighter  forms  may  entail  serious 
consequences,  since  many  effects  of  the  disease,  especially  the  damage 
to  the  spinal  cord  itself,  tend  to  increase  by  the  myelitic  tendency 
wliich  is  set  up.  The  neuralgic  pains,  which  result  from  the  damage 
to  the  nerve-roots,  are  extremely  obstinate.  But  in  many  cases  the 
symptoms  ultimately  pass  away — the  irritation  ceases  and  the  com- 
pressed structures  slowly  regain  their  function.  The  prognosis  is 
most  favorable  in  the  cases  which  result  from  injury  and  from 
syphilis.  But  in  all  cases,  even  those  of  syphilitic  origin,  in  which 
there  is  reason  to  infer  considerable  formation  of  new  tissue,  the 
prognosis  must  be  cautious,  since  the  cicatricial  contraction  of  this 
tissue  may  perpetuate  the  damage  to  cord  and  nerves  which  its 
pressure  has  produced.    The  prognosis  is  better,  ia  other  cases,  in 


INTERNAL    MENINGITIS.  333 

proportion  to  tlie  general  health  of  the  patient,  to  the  moderate 
degree  of  the  mischief,  and  to  the  extent  to  which  its  causes  are  under 
control. 

Treatment. — Eest  is  essential ;  in  severe  cases  it  should  be  abso- 
lute, and  in  every  case  movements  which  increase  pain  should  bo 
avoided.  The  pain  is  the  expression  of  irritation,  which  tends  to 
maintain  the  morbid  process.  Measures,  previoxisly  ineffective,  will 
often  be  followed  by  improvement  if  rest  is  added.  Posture  is  also 
of  great  importance.  The  prone  couch,  impracticable  in  acute  menin- 
gitis, can  often  be  employed  in  chronic  cases  with  advantage,  to  lessen 
mechanical  congestion  of  the  spine.  Warm  baths  and  diaphoretic 
baths  have  been  recommended  as  useful,  especially  in  subacute  cases. 
Next  in  importance  to  rest  and  posture  is  counter-irritation.  Repeated 
sinapisms  or  stimulating  liniments  may  be  used  if  mild  coimter-irrita- 
tion  only  is  required,  but  in  most  cases  greater  good  wiU  result  from 
more  energetic  means — blisters  or  the  actual  cautery.  The  last  is 
especially  recommended  by  Joffroy  for  the  hypertrophic  pachymenin- 
gitis. It  is  probable  that  posture  favours  the  beneficial  influence  of 
counter-irritation  in  a  very  important  degree. 

Sedatives  are  usually  needed  to  relieve  the  pain — morphia,  chloral, 
Indian  hemp  by  the  mouth,  subcutaneous  injections  of  morphia  or 
cocaine,  or  sedative  liniments  of  chloroform,  belladonna,  &c.  In 
employing  sedatives  it  must  be  remembered  that  they  will  usually 
be  needed,  in  chronic  cases,  for  many  months,  and  after  a  time,  by 
the  artificial  need  they  create,  they  may  apparently  keep  up  the  pain. 
It  is  therefore  well,  if  possible,  to  alternate  two  or  more  sedatives, 
to  make  cocaine  take  the  place  of  morphia  as  much  as  possible,  and  to 
lessen  the  dose  from  time  to  time  tentatively,  and  without  the  know- 
ledge of  the  patient. 

Iodide  of  potassium  and  mercury  are  the  agents  that  have  most 
influence  over  the  morbid  process  itself.  Both  are  effective  in  syphi- 
litic cases,  but  in  other  forms  of  the  disease  mei'cury  has  far  more 
influence  than  iodide  ;  it  may  be  rubbed  in  over  the  spine,  as  recom- 
mended in  the  acute  form.  The  ointment  and  oleate  may  be  rendered 
counter-irritant,  if  necessary,  by  the  addition  of  cantharides  ointment. 
In  many  cases,  tonics,  as  iron,  quinine,  and  cod-liver  oil,  are  needed. 
The  muscular  wasting  which  results  from  damage  to  the  nerve-roots 
requires  treatment  by  galvanism,  rubbing,  and  by  passive  movement, 
to  prevent  contractures.  If  hsemorrhagic  pachymeningitis  is  suspected, 
ergotine  may  be  used  in  addition  to  the  measures  suited  for  the  ordi- 
nary form.  Treatment  appears,  however,  to  exert  little  influence 
upon  it. 

Other  measures  are  the  same  as  have  been  described  for  the  acute 
form. 


334  SPINAL  MEMBJiANES, 


mEMOEEHAGB    INTO   THE    SPINAL   MEMBRANES; 
BL^MATOEACHIS. 

Spinal  hgeTnorrhage  maj  be  outside  tlie  dura  mater,  between  it  and 
the  bones  (extra-meningeal) ,  or  within  the  dura  mater  (intra-menin- 
geal).  In  the  latter  situation  the  extravasation  may  be  subdural, 
between  the  dura  mater  and  arachnoid,  or  it  may  be  subarachnoid, 
between  this  and  the  pia  mater.  Haemorrhage  outside  the  dura 
mater  is  more  common  than  within  it. 

Causes. — Meningeal  haemorrhage  may  occur  at  all  ages.  It  is  met 
with  in  newly-born  children,  but  in  them  is  due  to  rupture  of  vessels 
during  birth,  and  the  blood  often  merely  descends  into  the  spinal 
membranes  from  those  of  the  brain.  Spontaneous  haemorrhage  is 
unkuown  in  early  childhood,  but  occurs  at  all  other  ages,  and  is  more 
common  in  men  than  in  women.  Of  the  immediate  causes,  injury  is 
the  most  frequent — fractures  of  the  spinal  column,  blows  or  falls  on 
the  back  which  do  not  fracture  the  vertebrae,  and  even  falls  on  the 
feet  or  buttocks.  It  is  occasionally  found  after  death  from  severe 
convulsions,  epilepsy,  puerperal  eclampsia  (in  which  an  altered  state 
of  the  blood  may  assist),  chorea,  strychnine  poisoning,  and  tetanus. 
In  some  instances  no  symptoms  were  observed  during  life  which 
could  be  referred  to  the  haemorrhage,  and  it  is  probable  that  the 
extravasation  occurred  only  during  the  last  moments  of  life.  Hence 
it  seems  likely  that  muscular  spasm,  resulting  from  a  meningeal 
haemorrhage,  may  sometimes  have  been  looked  on  as  primary  and 
independent — the  cause  of  the  haemorrhage  when  it  was  really  the 
effect  of  it.  This  seems  certain  in  many  cases  of  tetanoid  spasm 
in  newly-born  children — in  most  cases,  indeed,  in  which  such  spasm 
occurs  almost  immediately  after  birth  and  is  associated  with"  menin- 
geal haemorrhage.  Such  cases  should  be  separated  from  those  to 
which  the  term  tetanus  neonatorum  is  applied.  They  are  further  con- 
sidered in  the  account  of  haemorrhage  into  the  cerebral  membranes  in 
Vol.  H.  Severe  and  prolonged  muscular  exertion  has  been,  in  a  few 
cases,  the  apparent  cause  of  spontaneous  haemorrhage.  It  also  occurs 
in  some  diseases  in  which  there  is  a  haemorrhagic  tendency,  such  aa 
purpuric  states,  and  the  haemorrhagic  forms  of  some  acute  specific 
diseases,  smallpox,  yellow  fever,  &c.,  and  very  rarely  in  typhoid  fever 
apart  from  any  haemorrhage  elsewhere.  In  most  of  these  cases  its  oc- 
currence has  not  been  suspected  until  the  post-mortem  examination 
revealed  it,  and  it  probably  occurs  generally  during  the  last  moments 
of  life.  A  rare  cause  is  the  bursting  of  an  aortic  aneurism  into  the 
spinal  canal,  after  erosion  of  the  bodies  of  the  vertebrae,  the  blood 
being  effused  outside  the  dura  mater.  Haemorrhage  within  the  dura 
mater  has  also  restilted  from  the  rupture  of  an  aneurism  of  a  vertebra] 


HEMORRHAGE.  835 

arterj.  Blood  may  descend  into  tlie  spinal  membranes  from  the 
cranium,  in  cases  in  wMcli  a  r-erebral  extravasation,  especially  about  tlie 
pons,  escapes  into  the  meninges ;  this  may  occur  in  adults  as  well  as 
in  the  meningeal  haemorrhage  that  is  produced  during  birth.  Lastly, 
in  cases  of  intense  meningeal  inflammation,  ecchymoses,  and  some- 
times considerable  extravasations,  have  been  found  on  both  sides  of 
the  dura  mater,  or  in  the  pia  mater. 

Pathological  Anatomy. — Extra-dural  hsemorrhage  comes  from 
the  plexus  of  large  veins  which  lie  between  the  dura  mater  and  the 
bone.  It  is  usually  not  of  large  extent,  and  the  blood  collects  chiefly 
on  the  posterior  aspect,  where,  in  the  recumbent  posture,  gravitation 
favours  accumulation,  and  the  space  between  the  membrane  and  the 
bone  is  greater  than  in  front.  But  sometimes  the  extravasation  is 
very  extensive,  covering  a  large  part  of  the  dura  mater,  and  in  such 
cases  it  may  extend  through  the  intervertebral  foramina,  along  the 
nerves.  Haemorrhage  is  more  common  in  the  cervical  region  than 
elsewhere,  but  may  occur  in  any  part.  The  blood  is  usually  coagu- 
lated, wholly  or  partially.  The  dura  mater  is  blood-stained,  some- 
times even  through  to  the  inner  surface.  The  spinal  cord  may  be 
compressed,  but  the  amount  of  blood  is  not  often  sufficient  for  this. 

It  is  easy  to  fall  into  grave  error  regarding  the  presence  of 
meningeal  hsemorrhage ;  when  a  body  has  been  lying  on  the  back,  the 
veins  outside  the  dura  mater  become  distended  with  blood,  and  this 
escapes  when  their  walls  are  divided  in  opening  the  sj)inal  canal. 
Care  must  be  taken  to  avoid  the  error,  on  the  one  hand,  of  regarding 
the  blood  thus  escaping  as  an  ante-mortem  extravasation,  and,  on  the 
other,  of  overlooking  a  hsemorrhage  which  actually  exists.  In  all 
cases  of  acute  spinal  disease,  in  which  a  post-mortem  examination  is 
to  be  made,  the  body  should  at  first  lie  face  downwards. 

Intra-meningeal  hsemorrhage. — An  extravasation  into  the  sac  of  the 
dura  mater  (subdural  haemorrhage)  may  be  small  in  quantity,  or  may 
fill  the  whole  cavity.  When  small  it  may  pass  from  one  part  to 
another.  In  subarachnoid  hsemorrhage,  the  blood  comes  usually  from 
the  vessels  of  the  pia  mater ;  it  may  surround  the  cord  for  an  mch 
or  two,  or  may  fill  the  whole  subarachnoid  cavity.  Such  extensive 
effusions  are  rare,  except  when  the  blood  descends  into  the  arachnoid 
from  the  cerebral  membranes.  Blood  effused  into  the  spinal  arachnoid 
has  been  known  to  ascend  as  high  as  the  pons,  and  even  to  break 
through  the  valve  of  Vieussens  and  get  into  the  cerebral  ventricles.* 
Simultaneous  cerebral  and  spinal  lisemorrhages  have  been  known  to 
occur  from  a  common  and  powerful  cause,  as,  for  instance,  in  a  case 
of  puerperal  eclampsia  (Charrier). 

In  meningeal  haemorrhage,  cerebral  as  well  as  spinal,  the  spinal  fluid 
is  often  blood-stained,  and  thus  spinal  hsemorrhage  may  be  thought 
to  be  more  considerable  than  it  really  is.     The  spinal  cord  is  often 

*  A«  in  an  apparently  conclusive  case  reported  by  Leprestre,  '  Arch.  gen.  de 
Med.,'  xxii,  p.  331. 


336  SPINAL    MEMBRANES. 

discoloured  and  compressed,  and  is  especially  damaged  wlieii  tlie 
Laemorrliage  is  beneath  the  araclmoid.  In  extensive  eztra-meningeal 
hsemorrliage,  the  subarachnoid  fluid  may  be  driven  away  from  the 
compressed  part,  and  may  distend  the  arachnoid  beyond  the  area  of 
compression,  the  limit  of  which  is  thus  marked.  In  cases  which  liave 
lasted  more  than  a  few  days  there  are  usually  signs  of  meningitis,  set 
up  by  the  irritation  of  the  blood. 

Symptoms. — As  already  stated,  slight  meningeal  hsemorrhage  is 
sometimes  found  post  mortem  in  death  from  convulsive  diseases  when 
no  symptoms  of  it  were  observed  during  life,  and  in  these  cases  it  is 
probable  that  the  extravasation  occiirs  during  the  lethal  convulsions. 
In  the  cases  in  which  hsemorrhage  causes  symptoms,  these  are,  for  the 
most  part,  those  of  meningeal  irritation,  and  thus  bear  some  resem- 
blance to  the  symptoms  of  meningitis.  They  differ  in  the  suddenness 
and  violence  of  their  onset,  to  which  exceptions  are  extremely  rare. 
These  symptoms  are  nearly  the  same  whether  the  haemorrhage  is  out- 
side or  inside  the  dura  mater. 

The  first  indication  of  the  lesion  is  usually  sudden  and  violent  pain 
in  the  back,  corresponding  in  position  to  the  seat  of  the  hsemorrhage, 
but  generally  felt  along  a  considerable  extent  of  the  back,  and  often 
severe  in  the  loins.  This  pain  in  the  back  is  usually  accompanied  by 
pain  along  the  course  of  the  nerves  passing  through  the  membrane 
near  the  extravasation,  darting  or  burning  pains,  often  of  great  in- 
tensity ;  they  are  paroxysmal  in  character,  and  between  the  pains  there 
maybe  various  abnormal  sensations,  tingling,  &c.,  referred  to  the  same 
parts,  and  accompanied  by  hypersesthesia.  Muscular  spasm  usually 
coincides  with  the  pain,  and  involves  partly  the  vertebral  muscles, 
causing  rigidity  of  the  spine  or  actual  opisthotonos,  partly  the  muscles 
supplied  by  the  nerves  in  which  the  pain  is  felt,  partly  the  muscles 
supplied  from  the  cord  below  the  seat  of  the  hsemorrhage.  The  con- 
vulsive movements  are  sometimes  general.  Intense  pain  in  the  back 
and  general  convulsion  have  been  known  to  be  the  only  symptoms. 
Occasionally  there  is  persistent  contraction  of  muscles,  and  there  is 
usually  spasmodic  retention  of  urine.  These  symptoms  of  irritation 
are  no  doubt  due  in  part  to  the  irritation  of  the  membranes  (causing 
the  vertebral  pain  and  reflex  spasm) ,  and  partly  to  the  irritation  of  the 
nerve-roots,  motor  and  sensory.  Paralytic  symptoms  quickly  follow — 
weakness  and  lessened  sensitiveness  in  the  limbs  below  the  lesion.  In 
some  cases  there  is,  at  last,  complete  loss  of  motion  and  sensation  in 
the  lower  limbs,  but  such  absolute  paralysis  is  not  common.  Loss  of 
power  at  the  onset  of  the  symptoms  usually  indicates  either  simul- 
taneous hsemorrhage  into  the  cord  or  the  effusion  of  a  very  large 
amount  of  blood.  It  occurs,  for  instance,  when  an  aneurism  bursts 
into  the  spinal  canal. 

The  symptoms  necessarily  differ  in  situation  according  to  the  seat  of 
the  disease.  When  hsemorrhage  is  in  the  cervical  region  (a  frequent 
seat),  the  pains  are  felt  in  the  neck  and  arms,  and  the  rigidity  may 


HEMORRHAGE.  337 

eanse  absolute  immobility  of  tbe  neck ;  while  dysphagia,  interference 
with  respiration,  and  dilatation  of  the  pupils  may  be  added  to  the 
other  paralytic  symptoms.  When  it  is  in  the  dorsal  region,  intense 
pain  encircles  the  chest  or  abdomen.  If  ia  the  lumbar  region,  the 
pain  is  felt  in  the  legs,  and  there  are  early  paraplegic  symptoms,  with 
loss  of  reflex  action  in  the  legs,  and  the  paralytic  incontinence  of 
urine  and  faeces  described  on  p.  246. 

As  a  rule,  in  spinal  haemorrhage,  the  cerebral  functions  are  un- 
afEected;  the  unfortunate  patient  is  conscious  of  all  his  sufferings 
from  first  to  last.  Occasionally,  however,  consciousness  is  lost  for  a 
short  time,  apparently  from  shock,  and  delirium  or  coma  may  come 
on,  either  as  an  indirect  effect  of  the  spinal  lesion  on  the  brain,  or  in 
consequence  of  a  sudden  increase  in  the  intra-cranial  pressure,  due  to 
the  displacement  upwards  of  the  cerebro-spiaal  fluid.  But  in  most 
cases  in  which  cerebral  symptoms  coexist  with  those  of  spinal  haemor- 
rhage, the  former  have  been  due  to  simultaneous  intra-cranial  disease ; 
this  is  the  case  when  the  blood  found  in  the  spinal  canal  comes  from 
within  the  skull,  as  in  most  cases  of  meningeal  haemorrhage  in  newly- 
born  children  (see  Vol.  II).  In  very  rare  cases  the  symptoms  of  spinal 
haemorrhage  have  come  on  insidiously,  without  paia,  as  in  a  case  in 
which  extensive  haemorrhage  outside  the  dura  mater  in  the  cervical 
and  upper  dorsal  region,  in  a  girl  of  fourteen,  caused  only  very 
gradual  weakness  in  the  arms,  and  difficulty  of  breathing,  from 
which  she  died  at  the  end  of  a  fortnight.*  Such  a  case  illustrates 
the  almost  universal  law  that  there  is  hardly  any  "characteristic" 
symptom  of  a  disease  that  is  not  sometimes  absent,  and  that  the 
absence  of  such  symptoms  has  incomparably  less  significance  than 
fheir  presence. 

The  symptoms  of  meningeal  haemorrhage,  in  acute  cases,  usually 
reach  their  height  in  two  or  three  hours,  but  sometimes  not  for  a  few 
days.  Death  may  occur  when  the  effects  are  fully  developed;  or, 
after  the  symptoms  have  reached  their  height,  they  may  decline,  to  be 
renewed,  a  day  or  two  later,  by  secondary  meningitis.  This  is  accom- 
panied by  some  pyrexia,  and  may  cause  death ;  but  if  moderate,  the 
symptoms  of  inflammation  last  a  week  or  ten  days,  and  then,  in 
favorable  cases,  permanent  improvement  commences. 

The  period  of  the  disease  at  which  death  is  most  common  is  a  few 
hours  after  the  onset  of  the  symptoms.  It  may  be  due  to  exhaustion, 
in  consequence  of  the  violence  of  the  pain  and  spasm,  but  is  more 
often  caused  by  the  interference  with  respiration  that  results  from  the 
conjoined  effect  of  the  palsy  and  spasm. 

Diagnosis. — In  cases  in  which  definite  symptoms  are  produced, 
the  diagnosis  rests  on  the  combination  of  sudden  local  pain  in  the 
back,  with  the  other  evidence  of  irritation  of  the  membranes,  nerve- 
roots,  and  cord,  above  enumerated.  When  the  haemorrhage  is  of 
traumatic  origin,  the  fact  that  the  symptoms  rapidly  followed  an 
*  R.  Jackson,  'Lancet,*  1869,  p.  5. 
VOL.   I.  22 


338  SPINAL    MEMBRANES. 

injury  facilitates  tlie  diagnosis.  In  the  extremely  rare  cases  in  wiich 
the  symptoms  come  on  insidiously,  without  pain,  the  diagnosis  of 
the  exact  nature  of  the  disease  is  scarcely  possible. 

In  haemorrhage  into  the  substance  of  the  cord,  vertebral  ptiin  is 
much  more  frequently  absent  than  in  meningeal  haemorrhage,  and 
when  it  occurs  is  less  intense  and  does  not  spread.  The  symptoms  of 
injury  to  the  cord  itself  are  prominent  from  the  commencement ;  there 
is  sudden  paralysis,  it  may  be  at  first  partial,  and  rapidly  increasing  ; 
even  if  there  is  some  spinal  pain  at  the  onset,  the  loss  of  power  is 
soon  recognised,  not  being  concealed  either  by  the  severity  of  the  pain 
or  by  spasm.  In  meningeal  hsemorrliage,  severe  pain  and  symptoms 
of  irritation  usually  precede  considerable  paralysis.  .  In  cases  which 
recover,  the  paralytic  symptoms  are  more  persistent  when  the  haemor- 
rhage is  into  the  substance  of  the  cord.  But  haemorrhage  into  the 
cord  often  breaks  through  into  the  membranes,  and  the  symptoms  of 
both  lesions  are  then  combined. 

Meningitis  is  distinguished  by  the  more  gradual  onset  of  the 
symptoms  and  by  the  presence  of  fever  from  the  first ;  the  fact  that 
inflammation  results  from  haemonhage  must  be  kept  in  mind.  In 
myelitis,  pain  is  commonly  absent,  and  the  symptoms  of  irritation  are 
insignificant.  The  diagnosis  from  tetanus  depends  on  the  absence  Of 
trismus,  on  the  presence  of  severe  spinal  pains,  and  on  the  sudden 
onset.  In  newly-born  children  the  diagnosis  is  supposed  to  be  difO.- 
cult,  but  during  the  first  few  hours  or  days  the  tetanoid  symptoms 
which  sometimes  occur  are  the  restdt  of  meningeal  haemorrhage  and 
injury  to  the  motor  cortex. 

One  case  of  spinal  haemorrhage  has  been  recorded  in  which  the 
eymptoms  very  closely  simulated  those  of  strychnine  poisoning. 
Yiolent  paroxysms  of  muscular  spasm,  with  intense  general  pain,  but 
without  spinal  pain,  followed  by  death  in  two  hours,  were  apparently 
due  to  an  extensive  haemorrhage  into  the  sac  of  the  dura  mater.* 
Analysis  revealed  no  strychnia  in  the  stomach.  In  such  a.  case  the  dia- 
gnosis would  have  to  be  determined  as  much  by  circumstantial  evidence 
as  by  the  symptoms,  and  even  the  post-mortem  discovery  of  haemor- 
rhage could  only  be  received  as  evidence  of  the  cause  of  the  convulsion 
if  no  other  were  found. 

Pbognosis. — In  all  severe  cases,  death  in  a  few  hours  is  to  be 
feared.  The  prognosis  only  becomes  less  grave  when  the  symptoms 
have  distinctly  reached  their  height,  although  there  is  still  some 
■danger  until  the  period  of  secondary  inflammation  is  over.  Paralytic 
symptoms  often  persist  for  some  time,  but  even  these,  in  the  end,  may 
pass  away.  At  the  onset,  the  prognosis  must  be  governed  by  the 
rapidity  with  which  the  symptoms  develop  and  by  the  seat  of  the 
disease.  Haemorrhage  in  the  cervical  region  is  especially  serious, 
ibecause  the  mechanism  for  breathing  is  directly  involved. 

Treatment. — The  first  point  is  to   secure   absolute  rest.      Even 
•  Dixon,  *  Lancet,'  1879,  p.  333. 


AN^MTA.  839 

passive  movement  siould  be  avoided.  Posture  is  also  of  most  Tirgeiit 
importance.  It  should  be  on  tbe  face  or  side,  and  not  on  tbe  back,  so 
that  the  spine  may  not  be  the  lowest  part.  The  prone  position  for  a 
few  hours  adds  greatly  to  the  chances  of  less  damage.  Yenesection 
has  been  employed  in  strong  individuals,  with  the  object  of  arresting 
haemorrhage  by  rapidly  lowering  the  blood-pressure.  Leeches  or  wet 
cupping  to  the  spine,  or  leeches  to  the  anus,  have  also  been  recom- 
mended in  cases  in  which  venesection  is  unadvisable.  It  is  probable 
that  the  local  abstraction  of  blood  does  more  in  this  than  in  most 
internal  haemorrhages,  on  account  of  the  communication  between  the 
venous  plexuses  and  the  veins  in  the  retro-vertebral  tissues ;  and  it  is 
probable  that  the  wisest  treatment  at  the  onset  would  be  scarification 
beside  the  spine,  opposite  the  seat  of  pain,  the  blood  being  allowed  to 
flow  freely.  This  may  be  followed  by  the  application  of  ice  and  by  the 
administration  of  ergotine  by  the  sMn  or  mouth.  Afterwards  the 
bowels  should  be  freely  opened.  Sedatives  are  usually  required  to 
relieve  the  pain.  The  stage  of  meningitis  must  be  treated  on  the 
principles  laid  down  for  the  management  of  the  acute  form  of  that 
disease.  The  residual  palsy  requires  treatment  by  electricity,  &c.,  in 
the  same  manner  as  the  consequences  of  meningitis. 

In  extra-men  i ngeal  haemorrhage  it  seems  justifiable  to  remove  blood 
that  is  compressing  the  cord,  by  opening  the  canal  and  perhaps  even 
washing  out  the  canal  with  an  astringent  and  antiseptic  solution. 
Even  when  the  blood  is  within  the  dura  mater,  the  operation  seems  to 
deserve  consideration  if  life  is  threatened. 


DISEASES  OF  TBE  SPINAL  COBD, 


ANEMIA  AND  HYPEREMIA  OF  THE   SPINAL   CORD. 

The  condition  of  the  vessels  of  the  spinal  cord  after  death,  their 
fulness  or  emptiness,  affords  no  indication  whatever  of  their  state 
during  life.  Inferences  as  to  the  ante-mortem  state,  drawn  from  the 
post-mortem  condition,  are  altogether  erroneous,  with  the  rare  excep- 
tion of  local  change,  in  which  the  vascular  condition  of  one  part 
differs  from  the  rest.  Practically,  such  local  variation  occurs  only  as 
part  of  a  local  lesion,  as,  for  instance,  in  the  hyperaemia  that  attends 
inflammation,  and  the  anaemia  that  results  from  pressui'e.  Hence  the 
occurrence  of  variations  in  the  state  of  the  vessels  of  the  cord,  and  the 
effects  that  such  variations  may  produce,  are  matters  of  inference  from 
symptoms  observed  during  life — symptoms  that  are,  in  themselves, 
open  to  various  interpretations.  Where  the  ground  is  barren  of  facts, 
theory  is  always  luxuriant.     Anaemia  or  congestion  of  the  cord  affords 


340  SPINAL    CORD. 

a  ready  explanation  of  symptoms  the  cause  of  "wliicli  is  unknown,  and 
it  is  scarcely  surprising,  therefore,  that  such  an  explanation  has  been 
often  given.  But  the  extent  to  which  the  conditions  have  been  invoked 
transcends  any  measure  of  justification,  and  surprise  cannot  but  be 
felt  at  the  absolute  confidence  and  precision  of  detail  with  which  these 
states  have  been  assumed  to  exist,  when  there  has  been  no  tittle  of 
definite  evidence.  Positive  assertions  always  receive  some  credence, 
however  unwarranted  the  assertions  may  be,  and  positions  incapable 
of  proof  are  sometimes  also  incapable  of  disproof.  It  would  be  a  futile 
and  useless  task  to  attempt  to  refute  in  detail  the  various  statements 
that  have  been  made  regarding  the  influence  of  ansemia  and  hyperaemia 
of  the  spinal  cord.  It  will  be  sufficient  to  point  out  briefly  what  may 
be  reasonably  surmised  regarding  these  morbid  states. 

Anjemia  of  the  Cobd. 

Permanent  diminution  in  the  tlood-swpply  to  considerable  areas  of 
the  spinal  cord  must  be  a  result  of  the  general  narrowing  of  the 
arteries  that  has  been  met  with  in  some  cases  of  chronic  meningitis ; 
but  it  is  then  associated  with  structural  changes  in  the  organ,  the 
effects  of  which  obscure  any  symptoms  that  can  be  ascribed  to  the 
anaemia.  Nothing  is  practically  known  of  the  special  consequences  of 
this  condition.  The  same  statement  may  be  made  of  the  arrest  of  the 
blood-supply  to  limited  areas,  although  it  may  safely  be  asserted  that 
loss  of  function  of  the  part  must  be  a  necessary  consequence.  This 
has  been  also  demonstrated  by  experiment.  Suddenly  produced 
anaemia  at  once  abolishes  function  and  quickly  causes  necrosis  of  the 
nerve-elements — some  of  which,  however,  break  up  less  rapidly  than 
others,  and  retain  for  a  longer  time  the  power  of  resuming  function 
if  the  blood-supply  is  restored.  It  is  possible  that  such  local  arrest 
of  the  circulation  is  the  initial  process  in  some  organic  lesions  of  the 
cord,  but  the  fact  has  still  to  be  demonstrated.  The  question  of  the 
occurrence  of  embolism  is  referred  to  in  a  separate  section. 

Transient  diminution  in  the  blood-supply  may  conceivably  be  the 
result  of  imperfect  embohsm,  but  has  not  been  proved  to  have  this 
origin.  It  is  sometimes  supposed  to  be  due  to  arterial  spasm,  de- 
pendent on  the  vaso-motor  nerves.  This  has  been  assumed  as  an 
explanation  of  certain  passing  symptoms  occasionally  complained  of, 
loss  of  power  in  the  legs,  and  sensory  disturbance,  tingling,  "pins 
and  needles,"  &c.,  in  the  legs  and  arms.  It  may  possibly  also  be 
the  cause  of  the  tetanoid  cramp  and  tinghng  in  the  hands  with  which 
patients  sometimes  wake  up  during  the  night.  The  theory  is  tenable, 
but  it  is  also  conceivable  that  such  symptoms  are  due  to  transient 
functional  states  of  the  nerve-cells  of  the  cord.  A  functional  de- 
rangement of  these  cells  must  be  the  immediate  cause  of  the 
symptoms,  whether  such  derangement  is  primary  or  is  produced  by 
spasm  of  the  vessels.     The  opinion  that  the  "  nocturnal  tetany,"  (as 


ANJIMIA.  341 

it  may  be  called,)  has  the  latter  origin  is  supported  by  the  fact  that 
the  occurrence  of  the  symptom  may  be  prevented  by  a  small  dose  of 
digitalis,  taken  at  bedtime. 

The  curious  symptom  known  as  "intermitting  lameness"  (which 
would  be  better  termed  "  paroxysmal  lameness ")  is  supposed  to 
depend  on  temporary  diminution  of  the  blood-supply  consequent  on 
arterial  degeneration,  but  it  is  not  known  whether  the  seat  of  this  is 
the  spinal  cord  or  the  brain.  The  character  of  the  symptom  suggests 
the  former,  but  its  associations  the  latter,  and  it  is  not  known  that 
the  arteries  of  the  cord  are  ever  the  seat  of  atheroma  in  the  cases  in 
which  this  condition  causes  the  cerebral  symptom  commonly  asso- 
ciated with  it,  "  dyslexia,"  a  peculiar  intermitting  difficulty  in  reading 
which  will  be  described  in  Yol.  II.  The  lameness  consists  in  a  sudden 
weakness  and  stiffness  of  one  leg,  accompanied  with  tingling  and 
blunting  of  sensibility,  and  of  pain  on  an  attempt  to  use  it.  The 
symptoms  are  increased  by  an  effort  to  continue  walking,  and  pass 
away  after  a  rest  of  ten  minutes  or  so,  usually  returning,  on  renewed 
exertion,  again  and  again.  The  difficulty  only  occurs  during  exertion. 
It  is  famiUar  to  veterinary  surgeons  as  occurring  in  horses.  The 
dyslexia  with  which  it  may  be  associated  is  of  grave  significance,  as  the 
cerebral  cause  seems  always  to  have  a  strongly  progressive  character. 

In  general  ansemia,  such  as  occurs  in  chlorosis  and  pernicious 
anaemia,  the  nutrition  and  function  of  all  organs  suffer,  and  the 
spinal  cord  shares  the  general  state.  The  readiness  with  which 
fatigue  of  the  legs  is  induced  in  such  conditions  may  be  due  to  the 
spinal  cord  as  well  as  to  the  muscles.  The  legs  often  ache,  and  are 
sometimes  the  seat  of  various  disordered  sensations.  It  is  probable 
that  these  are  the  result  of  the  impaired  nutrition  of  the  nerve- 
elements,  in  consequence  of  which  their  functions  are  readily  deranged. 
In  some  patients  graver  symptoms  occur, — weakness  of  the  legs,  some- 
times of  the  arms  also,  which  may  increase  slowly  to  complete  paialysis. 
SensPution  and  the  sphincters  may  also  be  affected.  The  symptoms  in 
these  cases  are  now  known  to  depend  upon  structural  changes  in  the 
spinal  cord,  to  which  reference  will  be  made  later.*  The  well-estab- 
lished fact  that  optic  neuritis  may  result  from  chlorosis  is  interesting 
as  proof  of  the  intensity  of  changes  in  nerve- structures  which  anaemia 
may  excite. 

In  the  quantitative  anaemia  that  results  from  loss  of  blood,  besides 
the  symptoms  just  described,  paraplegia  sometimes  comes  on,  under 
conditions  which  exclude  the  idea  of  hysteria.  The  source  of  the 
haemorrhage  has  most  frequently  been  the  stomach,  kidneys,  or 
uterus.  The  paralysis  is  usually  motor  only,  but  Ley  den  has  observed 
accompanying  hyperaesthesia.  It  may  come  on  a  few  hours  or  days 
after  the  loss  of  blood,  or  only  at  the  end  of  one  or  two  weeks.  Most 
cases  recover.  The  pathological  process  which  causes  the  paralysis  is 
not  known.  The  loss  of  power  is  comparable  to  the  loss  of  sight 
•  See  Section  on  "  Sclerosis  of  the  Cord  from  Toxic  Causes." 


342  SPINAL   CORD. 

wMcTi  occurs  from  the  same  catise,  in  which  there  may  he  no  visible 
morbid  change,  or  inflammation  may  be  found,  sometimes  succeeding 
the  loss  of  sight,  and  therefore  to  be  regarded  as  a  result  of  the 
derangement  of  the  nerve-elements,  or  of  the  influence  to  which  this 
is  due,  rather  than  as  the  cause  of  the  amaurosis. 

The  treatment  of  the  symptoms  due  to  general  ansemia  is  of  course 
essentially  the  improvement  of  the  blood-state.  Symptoms  which  can 
be  ascribed,  with  probability,  to  vaso-motor  spasm  may  be  prevented 
by  drugs  that  cause  a  more  uniform  arterial  tone,  such  as  digitalis  or 
belladonna,  coupled  with  nux  vomica  or  strychnia,  to  render  the  effect 
permanent. 

Hypee^mia.  of  the  Spinal  Coed. 

The  conditions  of  the  return  of  venous  blood  from  the  cord  probably 
shield  it  effectually  from  the  mechanical  congestion  from  which  almost 
every  other  organ  of  the  body  suffers  when  the  movement  of  blood 
through  the  thorax  is  hindered.  The  chief  mechanical  congestion  to 
which  the  cord  is  liable  is  that  which  results  from  the  influence  of 
gravitation,  and  occurs  when  a  person  is  lying  on  the  back.  The  dis- 
tension of  the  veins  outside  the  dura  mater,  and  also  of  those  of  the 
pia  mater,  which  occurs  when  the  heart  has  ceased  to  act,  and  the 
blood  is  free  to  obey  the  only  mechanical  force  which  then  acts  upon 
it,  sufficiently  proves  the  power  of  gravitation  to  congest  the  cord. 
But  it  is  not  probable  that  gravitation  has  anything  like  the  same 
influence  during  life,  counteracted,  as  it  then  is,  by  many  other  forces. 
Some  persons  with  imperfectly  nourished  cords  (easily  fatigued,  liable 
to  tingling,  &c.,  in  the  legs)  suffer  from  aching  in  the  legs  or  spine 
when  they  lie  on  the  back.  Since  this  ceases  when  the  posture  is 
changed,  it  is  probably  due  to  the  mechanical  congestion  of  the  cord 
or  membranes.  It  is  doubtful  whether  any  other  symptoms  can  be, 
with  confidence,  assigned  to  mechanical  congestion. 

Of  active  congestion  our  definite  knowledge  may  be  summed  up  in 
the  statement  that  it  occurs  in  the  early  stage  of  inflammation.  We 
know  nothing  of  it  as  an  independent  condition.  Nevertheless  many 
pages  might  be  filled  by  the  enunieration  of  the  various  symptoms  of 
congestion  of  the  spinal  cord, — in  which  the  unrestricted  play  of 
"  scientific  "  fancy  has  elaborated  a  symptomatology  for  the  separate 
congestion  of  every  part  of  the  spinal  cord.  Probably  the  only  case  ia 
which  we  are  warranted  in  suspecting  a  primary  active  congestion  of 
the  cord  or  membranes,  is  that  in  which  symptoms  identical  with  those 
of  commencing  myehtis,  and  following  a  cause  of  myelitis,  pass  away 
in  the  course  of  a  few  days.  These  cases  will  be  mentioned  in  the 
account  of  inflammation  of  the  cord. 

An  active  congestion,  which  may  be  called  secondary,  seems  to  result 
from  prolonged  and  violent  action  of  the  nerve-elements  of  the  spinal 
cord.     Thus  local  dilatation  and  distension  of  vessels,  migration  of 


MYELITIS.  343 

leucocytes  into  the  slieatli  and  into  tlie  adjacent  tissue,  and  even  small 
extravasations,  are  found  in  some  cases  of  hydrophobia,  of  tetanus, 
and  of  strychnine  poisoning,  and  it  is  probable  that  a  similar  con- 
gestion attends  all  violent  physiological  activity,  violent  and  continued 
musctdar  exertion,  coitus,  &c.  Coitus  several  times  repeated  has  been 
known  to  cause  haemorrhage  within  the  cord  which  must  be  preceded 
by  an  intense  congestion.  Vascular  dilatation,  with  an  increased 
blood- supply,  is  the  physiological  attendant  of  functional  activity  in 
all  organs,  and  doubtless  also  in  the  cord,  but  it  is  morbid  only  when 
excessive.  It  is  doubtful  whether  it  causes  symptoms  unless  it  goes 
on,  as  it  probably  may,  to  actual  inflammation. 


INFLAMMATION  OF  THE   SPINAL  CORD; 

MYELITIS. 

Inflammation  of  the  spinal  cord  is  a  common  lesion  in  the  acute, 
subacute,  and  chronic  forms.  It  usually  causes  a  considerable 
diminution  of  consistence,  and  hence  the  term  "  softening  of  the 
spinal  cord  "  has  been  used  as  a  synonym  for  it.  But  the  term  is  not 
accurate,  because  inflammation  does  not  always  cause  softening,  and 
a  diminution  of  consistence  may  occur  without  inflammation.* 

The  spinal  cord  differs  from  most  other  structures  in  that  its  power 
of  recovering  functional  capacity  after  damage  by  inflammation  is 
comparatively  small.  The  symptoms  persist  for  a  long  time,  and  are 
not  unfrequently  permanent.  Hence  the  distinction  between  acute 
and  chronic  inflammation  is  to  be  drawn,  not  from  the  whole  course 
of  the  disease,  but  from  the  mode  of  onset.  It  is  this  which  indicates 
the  character  of  the  morbid  process.  Acute  myelitis  is  that  form  in 
which  the  symptoms  indicating  inflammation  of  the  cord  come  on 
rapidly  and  reach  a  considerable  height  in  a  short  time,  in  less  than 
two  weeks.  When  the  onset  occupies  from  two  to  six  weeks  the 
inflammation  is  regarded  as  subacute.  When  a  stiU  longer  time 
elapses  before  the  symptoms  reach  a  considerable  degree  of  intensity 
the  myeUtis  is  termed  chronic,     "there  is,  of  course,  no  sharp  distinc- 

*  Oppenheim,  Pierre  Marie,  and  Bruns  have  been  especially  identified  with 
objections  which  have  been  raised  to  the  nomenclature  in  cases  of  myelitis.  They 
allege  that  many  cases  of  so-called  myelitis  are  due  to  softening  from  vascular 
thrombosis  and  to  other  causes  not  strictly  inflammatory  in  character.  They  even 
allege  that  compression  or  pressure  myelitis  is  a  misnomer.  There  is,  no  doubt,  much 
truth  in  what  they  allege  in  reference  to  softening,  but  it  is  not  easy  to  understand 
an  objection  to  naming  the  condition  myelitis  which  occurs,  at  least  in  many  cases 
of  compression,  and  the  views  as  to  what  constitutes  true  inflammation  are  so  con- 
flicting that  little  alteration  has  been  made  in  this  edition  in  the  point  of  view  from 
which  myelitis  is  regarded  in  earlier  editions  of  this  work. 


344  SriNAL    CORD. 

tion  between  these  classes.  The  subacute  and  acute  forms  maj  be 
conTeniently  described  together.  Other  varieties  are  based  on  the 
distribution  of  the  disease.  The  grey  matter  may  be  affected  only  or 
chiefly,  polio-myelitis  (from  iroXwi,  grey),  a  form  that  has  distinctiTe 
characters  which  make  its  separate  description  desirable.  When  the 
whole  thickness  of  the  cord  is  affected  in  a  small  vertical  extent  the 
myelitis  is  said  to  be  transverse.  This  is  one  of  the  most  common 
forms.  When  an  extensive  area  of  the  cord  is  continuously  inflamed 
the  myelitis  is  said  to  be  diffuse ;  when  one  small  area  is  affected  the 
inflammation  is  called /ocaZ;  when  there  are  many  foci  contiguous  or 
distant  the  myelitis  is  said  to  be  disseminated,  and  when  such  foci  are 
present  in  the  brain  and  pons,  as  well  as  in  the  cord,  the  condition  is 
spoken  of  as  disseminated  encephalo-myelitis.  Inflammation  of  the 
grey  matter,  around  the  central  canal,  extending  into  the  intermediate 
grej  substance  and  to  a  less  extent  into  the  cornua,  has  been  termed 
central  myelitis.  A  further  important  distinction  is  that  between 
jparenchymatous  myelitis,  an  acute  affection  beginning  in  the  nerve- 
elements,  and  the  interstitial  form,  which  begins  either  in  all  the 
elements  or  in  the  connecting  tissue,  and  in  which  vascular  disturbance 
takes  a  prominent  part.  The  latter  is  the  common  form  of  myelitis, 
whether  transverse  or  disseminated,  but  the  former  is  of  importance  on 
account  of  the  limited  class  of  symptoms  to  which  it  gives  rise  and 
of  its  special  causal  relations.  This  distinction  is  already  familiar  in 
regard  to  multiple  inflammation  in  the  nerves. 

Acute  Myelitis. 

ETiOLoaT. — Remote  causes. — Little  is  known  of  the  influence  of 
hereditary  predisposition,  but  myelitis  is  sometimes  met  with  in 
members  of  families  in  which  other  evidence  of  a  neuropathic  tendency 
can  be  traced.  Males  suffer  more  frequently  than  females.  It  may 
occur  at  all  ages,  but  the  form  which  occurs  in  young  children  is 
usually  confined  to  the  anterior  cornua ;  other  forms  of  myelitis  are 
most  common  between  ten  and  forty  years. 

Immediate  causes.— Myelitis,  acute  and  subacute,  is  readily  pro. 
duced  by  injuries  to  the  cord  of  all  kinds, — lacerations,  bruises,  and 
punctured  wounds.  It  also  seems  to  follow  simple  concussion,  but  in 
many  of  these  cases  there  have  been  minute  foci  of  structural 
damage.  The  myelitis  produced  by  concussion  is  often,  however,  far 
more  extensive  than  the  primary  lesion,  for  the  latter  may  cause  few 
symptoms,  and  a  few  days  later  a  complete-  transverse  myelitis  may 
develop.  Haemorrhage  into  the  cord,  especially  of  traumatic  origin, 
may  have  a  similar  effect ;  it  may  be  followed  after  a  short  interval 
by  extensive  myelitis.  In  cases  in  which  an  interval  of  more  than  a 
few  days  elapses,  pain  in  the  spine  may  show  that  a  morbid  process 
is  going  on  by  which  the  myelitis  is  produced.  Thus,  in  one  case  of 
acute  myelitis,  two  weeks   after  a  fall,  there  was  continuous"  pain 


ACUTE    MYELITIS.  S45 

during  the  interval,  corresponding  to  the  seat  of  the  later  inflamma- 
tion. Concussion  also  sets  up  the  subacute  form  of  myelitis,  and 
this  is  especially  common  after  railway  accidents  (see  Traumatic 
Lesions). 

AUied  to  injury  as  a  cause  is  violent  action  of  the  muscles  attached 
to  the  spinal  column,  which  has  sometimes  appeared  to  be  the  sole 
agent  in  producing  an  acute  inflammation  in  the  adjacent  part  of  the 
spinal  cord.  The  disease  has  been  known  to  follow  a  single  violent 
contraction  of  the  dorsal  muscles,  as,  for  instance,  a  successful  but 
violent  effort  to  prevent  a  fall.  It  is  possible  that  there  is  an  actual 
injury  to  the  cord  in  these  cases,  but  more  probably  inflammation  is 
set  up  in  the  fibrous  tissues  of  the  vertebral  column  or  nerve-roots, 
and  spreads  to  the  cord.  It  is  also  possible  that  compression  of  the 
vessels  may  be  concerned  in  the  mechanism.  In  several  cases  the 
disease  has  resulted  from  attempts  to  lift  heavy  weights.  Thus  a 
man,  two  days  after  such  an  attempt,  became  rapidly  paraplegic,  and 
ultimately  died ;  softening  of  the  cord  was  found  at  the  level  of  the 
fifth  and  sixth  cervical  vertebrae.  Slow  compression  (q.v.)  is  con- 
stantly attended  by  inflammation  in  the  compressed  part;  the 
myelitis  thus  set  up  may  have  an  independent  course,  acute  or 
subacute,  and  may  not  be  proportioned  to  the  pressure.  In  cancer 
of  the  vertebral  bones,  for  instance,  very  slight  pressure  may  cause  an 
acute  inflammation ;  there  is  probably  sometimes  an  extension  of 
inflammation  to  the  cord  from  the  external  disease,  but  such  extension 
is  not  necessary  for  the  occurrence  of  the  myelitis ;  the  inner  surface 
of  the  diira  mater  is  often  normal.  Acute  myelitis  also  results  from 
internal  meningitis,  in  which  some  degree  of  invasion  of  the  cord  is 
almost  invariable. 

Another  exciting  cause  is  exposure  to  cold,  especially  when  the 
body  is  heated,  lying  on  damp  grass  or  on  snow,  bathing  in  cold 
water,  &c.  This  cause  has  been  st^pposed  to  act  by  suppression  of  the 
functions  of  the  skin,  since  myelitis  has  been  produced  by  varnishing 
the  skin  of  animals,*  but  it  is  unlikely  that  this  is  the  sole  mechanism. 
Exposure  of  the  back  to  cold  sometimes  seems  especially  effective. 
Myelitis  has  been  produced  experimentally  in  rabbits  by  freezing  the 
skin  of  the  back  with  ether  spray  (Feinberg)  and  by  painting  the 
cord  itself  with  Fowler's  solution  (Leyden).  It  is  probable  that 
cold  usually  acts  by  the  mechanism  of  a  morbid  blood-state,  such  as 
that  which  causes  rheumatic  fever,  but  which  certainly  varies  greatly 
in  its  precise  character  and  pathogenetic  power.  It  is  also  certain 
that  local  exposure  frequently  causes  inflammation  in  organs  corre- 
sponding to  the  part  of  the  surface  that  is  especially  chilled,  although 
this  influence  may  be  chiefly  to  determine  the  incidence  of  the  effect 
of  the  blood-state.  In  myelitis  from  cold,  local  exposure  can  seldom 
be  traced,  and  the  common  mechanism  is  probably  that  of  a  blood- 

*  Peinberg,  '  Virchow's  Archiv,*  Bd.  lii.  In  Vulpian's  laboratory  similar  experi- 
ments failed. 


346  SPINAL   CORD. 

state.  It  is  instructive  to  compare  tlie  analogous  facts  regarding  tlwv 
causation  of  multiple  neuritis.  Orer-exertion  is  anotlier  powerful 
cause,  and  is  especially  effective  when  combined  with  insufficient 
food  and  exposure  to  cold. 

The  sudden  suppression  of  the  menses  and  of  other  habitual 
discharges  has  been  supposed  to  cause  the  disease.  In  most  instances 
of  the  kind  there  has  also  been  exposure  to  cold,  to  which  the  sup- 
pression and  the  myelitis  may  both  have  been  due,  and  it  is  impos- 
sible to  say  how  far  the  former  has  been  concerned  in  the  process. 
Thus,  a  girl  sat  on  wet  grass  during  menstruation,  which  was 
immediately  arrested,  and  myelitis  followed,  a  day  or  two  later.  It  is 
conceivable  that  the  sudden  arrest  of  the  discharge  may  lead  to  a 
morbid  state  of  the  blood,  or  modify  that  which  a  chill  alone  is 
capable  of  producing,  and  that  the  derangement  of  the  uterine  func- 
tions may  have  a  direct  influence  on  the  cord  and  dispose  it  to  suffer. 
It  is  noteworthy  that,  in  the  case  referred  to,  the  inflammation  was 
in  the  part  of  the  cord  to  which  the  pelvic  organs  are  related,  the 
lumbar  region. 

Functional  over-action  of  the  cord  may  cause  inflammation  in  the 
part  that  is  thus  over-exerted.  It  has  been  already  mentioned  that 
long-contiaued  stimulation  of  the  sensory  nerves  may  cause  myelitis 
at  the  spot  related  to  the  nerves  stimulated.  As  we  have  just  seen, 
active  function  everywhere  involves  vascular  dilatation,  which,  if 
excessive,  amounts  to  active  congestion,  and,  when  prolonged,  is 
attended  by  the  escape  of  leucocytes  that  renders  the  condition  one 
of  inflammation.  However  excited,  such  a  process  has  a  tendency 
to  independence  of  course,  and  to  proceed  beyond  the  degree  corre- 
sponding to  its  cause.  It  is  especially  local  functional  over-action 
that  has  a  tendency  to  cause  myelitis.  Thus  the  affection  seems  to 
be  sometimes  produced  by  sexual  excesses.  Occasionally,  however, 
general  over-action  of  the  cord,  as  in  severe,  long-continued  exer- 
tion, has  been  followed  by  the  disease.  In  one  case  it  was  due  to 
lying  at  rest  on  damp  ground,  after  an  imusuaUy  long  walk  on  a 
hot  day. 

Toxic  blood-states  constitute  a  well-marked  class  of  causes,  and 
their  influence  is  rendered  the  more  distinct  by  the  analogous  causa- 
tion of  multiple  neuritis.  They  may  be  acute  conditions  due  to  some 
organised  virus,  perhaps  allied  to  those  that  produce  the  acute  specific 
diseases.*  Inflammation  of  the  cord  occasionally  comes  on  in  the 
course  of,  or  after,  typhus,  typhoid,  erysipelas,  malaria,  and  especially 
variola  and  measles,  as  well  as  acute  rheumatism  and  severe  puerperal 
diseases.  The  onset  may  be  during  the  acute  disease,  at  any  period 
after  it  has  existed  for  about  a  third  of  its  duration,  or  it  may  occur 
during  convalescence,  or  a  month  or  more  after  the  disease  is  over. 

•  See  Buzzard  and  Risien  Russell  ('Trans.  Clin.  Soc.,'  1897),  who  describe  a  case 
of  meningo-myelitis  in  which  a  diplococcus  was  found  in  the  membranes,  and  caltures 
on  nutrient  media  showed  the  presence  of  an  identical  organism. 


ACUTE   MYELITIS.  347 

Whether  syphilis  is  a  cause  of  acute  myelitis  is  a  question  on  which 
opinions  differ.  Attacks  of  acute  myelitis  are  not  imcommon  in 
syphilitic  subjects,  and  it  seems  on  the  whole  probable  that  the 
mechanism  is  by  disease  of  the  vessels,  such  as  is  found  in  the  pia 
mater  in  some  cases.*  The  myelitis  that  occurs  in  acute  specific 
diseases  is  probably  due  to  the  virus  of  the  primary  disease,  to  a 
product  of  it,  or  to  a  secondary  infection  engrafted  on  the  first. 
Thus  Marinesco  found  streptococci  in  the  spinal  cord  in  a  patient  in 
whom  paralysis  came  on  in  the  course  of  smallpox.  The  fact  that 
the  inflammation  is  commonly  disseminated,  suggests  that  some 
specific  organisms  have  a  tendency  to  fix  themselves  in  the  spinal  cord 
and  set  up  inflammation.  In  diphtheritic  paralysis,  the  cells  of  the 
cord  may  present  changes  that  amount  to  a  parenchymatous  myelitis. 
The  fact  that  chronic  changes  in  the  cord  may  be  a  late  effect  of 
syphiHs  (as  in  tabes)  makes  it  the  more  easy  to  conceive  that  acute 
processes  should  sometimes  occur  in  an  earlier  stage  of  the  disease.f 

In  another  class  of  diseases,  a  special  influence  of  the  virus  appears 
to  be  associated  with  the  results  of  functional  over-action.  This 
effect  is  especially  seen  in  hydrophobia  and  in  tetanus.  In  some 
cases  of  this  disease,  foci  of  a  somewhat  diffuse  myelitis  of  slight 
degree  are  conspicuous  on  microscopical  examination.  Still  more 
intense  myeHtic  changes  are  sometimes  met  with  in  the  chorea  of  the 
dog,  although,  in  other  instances,  the  cord  is  normal. 

Chronic  blood-states  and  constitutional  conditions  may  also  cause 
acute  myelitis.  Alcoholism  is  an  occasional  cause,  and  frequently 
produces  the  subacute  form.  Gonorrhoea  has  also  been  shown  by 
Ley  den,  I  Gowers,§  Barrie,  and  others  to  be  a  cause  of  myelitis.  ||  The 
most  potent  agent  of  this  class  is  gout.  This  is  known  to  be  a 
frequent  ca,use  of  inflammation  in  various  structures, — of  the  nerves 
among  the  rest ;  and  cases  of  acute  and  subacute  myelitis  are  met 
with,  especially  of  the  disseminated  and  relapsing  forms,  for  which 
no  other  cause  can  be  discovered.  In  one  such  case  the  patient  had 
but  lately  recovered  from  a  severe  and  prolonged  attack  of  gouty 
inflammation  of  several  intra-ocular  structures.  Like  other  chronic 
general  causes,  gout  probably  often  acts  as  a  predisponent  when  the 
malady  is  excited  by  some  other  cause. 

Irritation  and  inflammation  of  peripheral  organs,  as  the  uterus, 
bladder,  kidneys,  intestine,  &c.,  have  been  supposed  to  be  an  occasional 
cause,  but  this  influence  is  exceedingly  doubtful,  except  in  the  rare 

•  Sudden  paraplegia  occurred  in  a  case  in  which  there  was  diffuse  arteritis,  sup- 
posed to  be  syphilitic  (Schmaus,  *Arch.  f.  kl.  Med.,'  1889);  see  also  Williamson, 
*  Lancet,'  1894. 

+  Bourget  ('  Aroh.  de  Med.  exper.,'  1893)  found  acute  myelitis  almost  destroying 
the  lumbar  enlargement,  produced  by  the  injection  of  the  streptococci  of  erysipelas. 

t  '  Zeitschr.  f.  kliii.  Med.,'  1892. 

§  '  Clinical  Lectures,' Gowers. 

II  Compare  with  this  a  condition  of  neuritis  resulting  from  gonorrhoea  (p.  147)* 


848  SPINAL    CORD. 

cases  in  wliicli  inflammation  of  a  nerve  ascends  to  tlie  cord.  In  a 
considerable  number  of  cases  we  are  able  to  discover  no  cause  to 
which  myelitis  can  be  ascribed.  Many  of  these  are  probably  due  to  a 
blood-state  which  may  have  no  other  consequences  or  may  also  cause 
simultaneous  inflammation  elsewhere.* 

Pathological  Anatomy. — ^In  recent  acute  inflammation  of  the 
cord,  the  pia  mater,  at  the  affected  part,  is  usually  red  and  vascular, 
and  the  cord  itself  may  be  slightly  swollen.  Its  tissue  is  reddened  and 
often  marbled  from  distended  vessels,  and  the  distinction  between 
white  and  grey  matter  is  indistinct  or  lost. 

The  diminution  of  consistence  which  occurs  in  acute  inflammation 
may  be  so  great  that  the  affected  part  is  diffluent,  like  cream,  and 
flows  out  when  the  pia  mater  is  divided.  It  does  not  follow  that  the 
consistence  is  lessened  to  this  degree  during  life ;  post-mortem  soft- 
ening occi^"s  more  readily  at  an  inflamed  part.  When  the  vascular 
disturbance  and  extravasation  are  considerable,  the  condition  is  termed 
"  red  softening ;"  so  much  blood  may  be  effused  into  the  tissue  that 
the  part  looks  as  if  there  had  been  an  actual  haemorrhage,  and  ia 
some  cases  an  extensive  extravasation  does  actually  exist ;  this  form 
is  termed  "  hsemorrhagic  myebtis  "  If  the  inflammation  has  existed 
for  some  time,  the  effused  blood  is  altered  in  tint,  and  the  colour  of 
the  softened  part  may  be  rather  chocolate  than  red.  After  a  longer 
time  the  changes  in  the  blood-pigment  cause  the  colour  to  be  lighter, 
"yellow  softening."  In  many  cases  the  breaking  up  of  the  nerve- 
elements  (partly,  as  just  stated,  post  mortem)  is  out  of  all  proportion, 
to  the  extravasation  of  blood,  so  that  the  softening  is  "  white."  It  is 
said  that  red  and  yellow  softening  may  ultimately  become  white  from 
the  removal  of  the  blood-pigment,  but  the  evidence  of  this  is  inade- 
quate. The  period  at  which  these  changes  of  colour  are  found  varies 
according  to  the  amount  of  extravasation  at  the  onset.  At  a  later 
period  the  yellow  or  white  opaque  aspect  is  changed  to  a  grey  trans- 
lucent appearance,  from  the  absorption  of  the  fatty  products  of 
degeneration,  and  an  increase  in  the  connective  tissue.  If  the 
myelitis  is  very  limited  in  extent,  cavities  may  be  formed  within  the 
cord. 

The  microscopical  appearances  in  myelitis  vary  much  according  to 
the  duration  and  form  of  the  inflammation.  In  the  fresh  state  the 
most  conspicuous  objects  are  the  products  of  degeneration  of  the 
fibres,  granules  and  masses  of  myelin,  and  granule  corpuscles,  with, 
in  older  cases,  the  peculiar  bodies  termed  "corpora  amylacea" 
(Fig.   97).       With  these  are  numerous  red  blood-discs,  leucocyte- 

*  Thoiiiot  and  Masselin  ('Rev.  de  Med.,'  1894)  injected  pure  cultures  of  StapTiy* 
lococcus  aureits  and  Bacterium  coli  commune  into  rabbits,  producing  paraplegia, 
the  result  of  spinal  cord  changes.  The  grey  matter  was  chiefly  affected,  and  the 
micro-organisms  could  be  found  in  the  cord  as  long  as  six  months  later.  They 
regard  so-called  reflex  paraplegia  as  almost  certainly  the  result  of  some  infective 
condition  similar  to  that  which  they  pi'oduced  artificially. 


ACUTE    MYELITIS. 


349 


like  corpuscles,  and,  after  the  first  week,  distinct  cells,  rotmd,  oval, 

spindle-shaped,  or  angular,  with  nuclei  which  resemble  leucocytes  in 

size,  and  are  readily  mistaken  for  them.     Fragments  of  axis-cylinders 

may  also  be  seen,  granular  in  aspect,  and  with  irregular  swellings 

(Fig.  97). 
The  softened  and  even  dif&uent  state  of  the  tissue  prevents  us  from 

learning  much,  by  microscopical  examination,  of  the  condition  of  the 

])arts  in  which  the  inflammation 

is  intense  and  diffuse,  because 

the  breaking  up  of   the   tissue 

and  separation  of  the  fragments 

renders  it  impossible  to  obtain 

sections  of  the  hardened  organ. 

It  is  only  where  the  inflamma- 
tion is  less  intense,  or  less  uni- 
formly diffused,  that  a  thorough, 

examination  can  be  made.     The 

products    of    degeneration    are 

best  seen  in  the  fresh  state  or  in 

sections  mounted  in    glycerine 

(Fig.  97).      The  fragments  and 

globules  of  myelin  in  part  occupy 

the  position  of  the  nerve-fibres 

from    which   they    have    come, 

in  part  are  aggregated  into 
masses. 

In  sections  rendered  trans- 
parent in  the  ordinary  way, 
the  fatty  products  of  the  de- 
struction of  the  nerve-elements 
are  invisible,  and  the  most  con- 
spicuous alterations  are  the  dila- 
tation of  vessels,  which  is  often 
very  great,  and  the  accumulation  of  leucocytes  and  other  cell- 
elements  in  the  tissue,  and  especially  about  the  vessels.  In  the  white 
substance  the  larger  vessels  are  conspicuous  by  their  enlargement, 
and  in  the  grey  substance  the  dilated  arteries  and  capillaries  may 
occupy  a  considerable  part  of  the  tissue  (Figs.  98,  99,  a).  The  nuclei 
of  the  capillaries  are  large  and  numerous.  The  walls  of  the  smaller 
arteries  are  encrusted  with  leucocytes  in  the  early  stage,  and,  later 
on,  in  both  white  and  grey  substance,  are  much  thickened  by  cells, 
the  nuclei  of  which  are  often  elongated  and  more  or  less  concen- 
tric to  the  lumen  of  the  vessel  (Fig.  102,  d).  Outside  this  thickened 
waU  the  perivasctilar  sheath  is  enormously  distended,  at  first  by  leuco- 
cytes, among  which,  afterwards,  other  cells  are  mingled — round, 
fusiform,  angular,  with  leucocyte-like  nuclei  (Fig.  102).  Bi  transverse 
section  this  distended  sheath  may  look,  at  first  sight,  l^e  an  enormously 


Fia.  97. — Myelitis,  a,  an  irregularly 
swollen  axis-cylinder.  5,  section  of 
nerve-fibre  with  swollen  axis-cylinder. 
c,  section  of  white  substance,  with 
granule-masses  and  atrophied  nerve- 
fibres,  d,  corpora  amylacea.  (Prom 
Leyden.) 


850 


SPINAL   CORD. 


thickened  wall.  Ked  blood-corpuscles  distend  the  vessels  and  are  seen 
also  in  the  adjacent  tissue,  sometimes  uniformly  scattered  through  it 
(Fig.  102,  f),  sometimes  aggregated  in  small  extravasations  due  to  the 
rupture  of  minute  vessels.  The  grey  substance  is  densely  set  with 
round  corpuscles,  staining  deeply,  many  of  which  are  the  nuclei  of 
small  fusiform  or  angular  cells  (Figs.  99,  a,  101,  b),  while  the  inter- 
vening substance  is  much  more  granular  than  in  health.     The  nerve- 


Fl<J.  98. — Subacute  myelitis,  lumbar  region;  both  white  and  grey  fiub- 
stance  uniformly  affected.  In  the  latter  the  numerous  distended  vessels 
appear  as  lines ;  here  and  there  a  larger  one  is  visible. 


'^^r-'-„   n,^' 


Fia.  99. — Portions  of  the  section  shown  in  the  last  figure,  more  highly  magnified. 
A,  from  the  left  ant.  coruuj  distended  capillaries  with  numerous  nuclei;  larger 
vessels  with  slight  distension  of  perivascular  sheaths;  numerous  round  and  stellate 
cells;  ganglion-cells,  swollen,  with  shrivelled  processes.  C,  from  front  of  post, 
column;  scattered  nerve-fibres  separated  by  amorphous  material  in  which  small 
cells  can  be  seen,  round  and  irregular,  some  distinct,  others  indistinct,  vessels 
with  thickened  walls.  B,  from  near  the  posterior  surface,  shows  similar  changes 
but  with  moi'e  open  spaces  from  which  nerve-fibres  have  perished.  (From  sectiona 
prepared  by  Dr.  Money.) 


ACUTE    MYELITIS.  851 

cells  are  mucli  swollen  and  granular,  often  contain  distinct  globules, 
strongly  refracting,  and  probably  fatty.  The  cell-processes  appear 
shrivelled  or  lost  (Fig.  99,  a).  In  slight  cases  the  margins  of  the 
cells  may  be  less  sharply  defined  than  in  health,  and  the  bodies  then 
may  contain  large  vacuoles,  sometimes  wholly  within  the  cells,  some- 
times partly  within  and  partly  outside  them  in  an  adjacent  albuminous- 
looking  substance.*  In  some  cases,  however,  especially  those  of 
disseminated  interstitial  myelitis  of  moderate  degree,  the  nerve-cells 
appear  little  changed  in  hardened  and  cleared  specimens,  although  a 
gramilar  condition  can  usually  be  seen  in  the  recent  state. 

In  the  white  substance,  cleared  sections  show  a  great  increase  and 
alteration  in  the  interstitial  tissue.  The  change  may  be  uniformly 
distributed,  or  greatest  in  the  neighbourhood  of  the  vessels  (Fig.  102, 
c).  The  increase  is  due  in  part  to  an  amorphous  material,  contain- 
ing nuclei  here  and  there,  some  of  which  belong  to  cells  such  as 
have  been  described  in  the  grey  substance ;  and  these  may  constitute 
extensive  tracts  between  the  spaces  where  nerve-fibres  once  existed 
(Fig.  102).  In  older  cases  the  tissue  may  have  a  fibrous  aspect  in 
places.  The  large  cells,  with  many  processes  called  "  spider  cells," 
or  "  cells  of  Deiters,"  are 
often     conspicuous     objects      S^w-'^'^x''-"^  T-' ."><^^^„.,.|^  ^,j^- ,« 


r 


(Fig.  100).      The    processes  '^^ 
extend    between    the    nerve- 
fibres.     "We  have  seen    that 

their  existence  in  the  normal  ^jy ^^    ^  -^                                           ^ 

cord  has  been  doubted,  but  in  ^&\^ '^%^*''^                                      - 

the  inflamed  cord,  in  which  ^0^^^^'^#^^'y-^\?-,y\"TL'                    ^ 

they  are  enlarged,  their  pre-  ^'^^h'^^'Q>^'^^^''^'i-^   "■  -1^.1^^-^            ^„    •^ 

sence  IS  distinct.  <^-^'.i        ^                              •>4»>»" 

The  changes  in  the  nerve- 
„,  I  mi,  ViQ.  100. — Subacute  disseminated   myelitis; 

fibres     vary     much.  Ihey  pan.  of  the  posterior  columu.     Nerve-fibres 

always       suffer       destructive  separated  by  granular  material  and  products 

changes,  but  the  products  of  of    degeneration    and   by    numerous    large 

°     '  ,^  spider  cells.      Ine  myelitis  in  this  case  was 

their      degeneration,       above  attended  by  optic  neuritis.     (From  a  see- 

described,  are  scarcely  to  be        tion  lent  by  Dr.  Dreschfeld.) 
seen    in    cleared    specimens. 

The  slightest  change  in  the  myelin  appears  to  consist  in  an  alteration 
in  its  chemical  nature,  in  consequence  of  which  it  stains  more  readily 
than  in  health.  Fibres  are  thus  changed  adjacent  to  any  increase 
in  the  interstitial  tissue,  and  they  contrast  with  the  unstained  healthy 
fibres  in  the  vicinity  (Fig.  101,  c).  With  or  without  this  change  in 
the  myelin,  there  is  an  irregular  swelling  of  the  axis-cylinders,  such  as 
is  seen  on  examination  in  the  fresh  state.     If  the  fibres  are  divided 

*  The  significance  of  this  vacuolation  has  been  much  discussed.  It  is  probable 
that  the  vacuoles  form  after  death,  perhaps  during  the  process  of  hardening,  but 
that  they  do  not  form  unless  the  cells  have  been  altered  by  disease.  They  have 
thus  some  pathological  significance. 


852 


SPINAL  COED. 


transversely  at  the  places  wliere  the  axis-cylinder  is  swollen,  this 
appears  much  larger  than  normal  (Fig.  97,  6).  Examples  of  this 
will  be  found  in  most  of  the  figures.  These  intense  degenerative 
changes  cause  the  breaking  up  of  the  myelin,  and  may  involve  the 
destruction  of  the  fibre,  the  space  occupied  by  it  being  left  empty. 
In  othe]  cases,  however,  the  axis-cylinder  persists  and  remains  sur- 


Flff.  101. — Acute  transverse  myelitis,  fatal  in  three  weel<s.  Carmine 
preparations.  A,  section  throug'h  most  diseased  part,  at  first  lumbar 
segment.  Foci  of  inflammation  scattered  through  the  whole  area  of  this 
cord,  most  abundant  in  the  posterior  columns  (the  apparent  enlargement 
of  which  is  probably  due  to  the  direction  of  the  section).  Dilatation  of 
the  central  canal.  B,  from  the  anterior  cornu  at  A,  S,  a  distended 
vessel;  the  tissue  crammed  with  lymphoid  and  other  cells,  among  which 
run  normal  nerve-fibres.  C,  from  the  posterior  column.  A,  c.  The  areas 
staining  deeply  are  seen  to  consist  of  tliickened  trabeculsa,  lymphoid 
cells,  amorphous  tissue,  and  also  of  nerve-fibres,  the  white  substance  of 
which  stains  with  carmine  while  that  of  the  neighbouring  healthy  fibres 
does  not.* 


•  I  am  indebted  to  Dr.  Dreschfeld  for  the  section  from  which  these  drawings  are 
made.  The  patient  was  a  girl  aged  twenty-three;  paralysis,  motor  and  sensory, 
was  complete  at  the  end  of  two  days;  the  application  by  her  mother  of  hot  bottles 
to  the  feet  caused  such  extensive  sloughing  ulcers  that  amputation  of  one  leg  waf 
deemed  nececsary  three  weeks  after  the  onset,  and  the  patient  died  the  next  day. 


ACUTE   MYELITIS.  853 

rounded  by  a  narrow  zone  of  myelin.  Often  a  large  ntunber  of  such 
skrunken  fibres  may  be  found  in  a  part  wbicli  at  first  sight  appeared 
devoid  of  fibres.  This  shrinking  of  the  fibres  occurs  not  only  as  the 
result  of  pressure  on  them  by  the  connective-tissue  elements,  but  also 
where  there  is  little  interstitial  change  and  no  compression. 

The  relative  amount  of  change  in  the  nerve-elements  and  in  the 
interstitial  tissue  varies  very  much.  In  some  cases  the  interstitial 
changes  are  evidently  primary,  and  the  fibres  at  first  present  little 
change  in  slight  cases,  but  afterwards  are  altered  secondarily,  being 
narrowed,  and  even  destroyed,  as  they  are  surrounded  and  enclosed 
by  the  products  of  the  inflammation.  In  other  places,  or  even  other 
parts  of  the  same  section,  there  is  but  little  increase  in  the  neuroglia, 
although  the  fibres  have  suffered  extensively ;  empty  spaces  are  limited 
by  septa  but  little  thicker  than  normal,  although  with  more  nuclei, 
aiid  often  with  numerous  granules,  the  nature  of  which  is  uncertain. 
Occasionally  there  is  a  more  diffuse  change  in  the  white  substance ; 
the  neuroglial  tissue  is  increased  by  small  ceUs  and  amorphous 
material  in  such  a  manner  that  all  appearance  of  septa  and  spaces 
is  lost ;  a  few  nerve-fibres  are  included  in  it,  and  the  spider  cells  are 
very  conspicuous  objects,  their  processes  apparently  representing  the 
septa  that  exist  in  the  normal  condition  (Fig.  100). 

The  distribution  of  these  changes  is  very  variable.  The  whole 
thickness  of  the  cord  is  uniformly  affected  in  pressure-myelitis  (see 
"  Compression  of  the  Spinal  Cord ").  In  transverse  myelitis  the 
changes  may  be  so  intense  in  all  the  structures  at  the  affected  level 
that  the  cord  is  diffluent,  and,  as  already  stated,  only  separated  pro- 
ducts of  degeneration  and  inflammation,  and  blood-corpuscles  are  to 
be  seen.  In  slighter  cases  the  alteration  is  seldom  distributed  uni- 
formly, and  in  either  a  single  section,  or  a  series  of  sections  taken 
near  together,  each  part,  may  present  variations  in  the  damage,  which 
may,  however,  be  such  that  no  part  escapes  in  all  (see  Fig.  102).  In 
meningo-myelitis  the  periphery  of  the  cord  is  always  most  affected, 
and  in  old  cases  the  thickened  pia  mater  may  send  tracts  of  dense 
tissue  into  the  interior  of  the  cord,  from  which  branching  processes 
of  tissue  may  pass  on  all  sides.  The  condition  is  similar  to  that  met 
with  in  chronic  myelitis.  A  similar  extension  is  often  conspicuous 
in  cases  in  which  there  is  no  special  inflammation  of  the  membranes, 
and  the  interstitial  changes  often  extend  from  the  septa  that  normally 
pass  into  the  cord  and  from  the  walls  of  the  vessels,  the  course  of 
which  may  thus  be  marked  out  when  the  vessels  cannot  be  seen. 
Hence  the  white  columns  may  be  broken  up  by  lines  of  tissue  passing 
from  the  surface  of  the  cord  towards  the  grey  substance. 

In  disseminated  myelitis  the  foci  of  inflammation  may  exist  through 
a  wide  extent  of  the  cord,  and  appear  on  the  surface  as  reddish-grey 
areas,  closely  resembling  those  of  disseminated  sclerosis,  but  less 
sharply  outlined.  Under  the  microscope,  in  the  early  stage,  smaller 
foci  stud  the  grey  and  white   substance  irregularly,  sometimes  ex- 

VOL.  I.  2'6 


354 


SPINAL    CORD. 
Fia.  102. 


'-'-^•'^   ■^■i      '    W.R.Gael    .sit J 


ACUTE    MYELITIS.  855 

tending  into  larger  tracts.  They  may  be  most  abundant  in  tlie  grey 
or  the  white  substance.  At  first  the  vascular  changes  are  the  more 
conspicuous,  but  afterwards  the  evidence  of  destruction  of  nerve- 
elements  and  the  inflammatory  products,  exudative  and  cellular, 
described  above,  characterise  these  foci,  while  ultimately  a  condition 
of  irregular  focal  sclerosis  remains.  In  central  myelitis  there  is  a 
great  increase  in  the  nuclei  about  the  central  canal,  with  vascular 
changes  in  the  commissure  adjacent,  but  there  may  also  be  evidence 
of  slight  widely  diffused  myelitis.  It  is  necessary  to  remember  that 
the  nuclei  around  the  central  tissue  vary  much  in  number,  and  that 
it  is  easy  to  mistake  a  normal  for  a  diseased  condition. 

Little  is  known  of  the  morbid  appearances  in  the  parenchymatous 
forms,  except  that  in  a  variety  of  anterior  polio-myelitis,  and  in 
diphtheritic  and  other  toxic  paralyses,  the  nerve-ceUs  present  granular 
changes,  and  the  interstitial  grey  substance  may  be  but  slightly 
affected. 

The  nerve-roots  coming  from  a  much  inflamed  part  are  -usually 
damaged,  and  may  present  histological  changes  similar  to  those 
within  the  cord — distension  of  vessels,  increase  of  nuclei  and  of  con- 
nective tissue  about  the  vessels,  breaking  up  of  the  myelin,  swelling  of 
the  axis-cylinders.  Degenerative  and  neuritic  changes  may  descend 
the  motor  nerves,  although  rarely  to  the  same  degree  as  in  polio- 
myelitis, except  in  the  cases  in  which  there  is  extensive  inflammation 
in  the  lumbar  or  cervical  enlargement. 

From  the  foci  of  inflammation,  ascending  and  descending  degenera- 
tions pass  along  the  tracts  of  long  fibres  that  have  been  already 
described,  downwards  in  the  pyramidal  tracts,  upwards  in  the  poste- 
rior median  columns,  the  direct  cerebellar  tracts,  and  the  antero- 
lateral ascending  tract.  In  cases  of  transverse  myelitis,  moreover, 
changes  that  are  definitely  inflammatory  in  nature  may  be  traced 
in  certain  tracts  for  a  short  distance  above  or  below  the  chief  lesion ; 
they  may  accompany  the  secondary  degeneration,  or  may  be  found 
extending  along  a  tract  that  degenerates  in  the  opposite  direction. 
Thus  distinct  inflammation  may  pass,  for  a  short  distance,  tip  the 

Fia.  102. — Acute  transverse  myelitis;  from  a  case  fatal  in  a  month.  A,  B,  C, 
neighboui-ing  sections  from  the  inflamed  part,  in  the  mid-dorsal  region,  to  show 
the  varying  distribution  of  the  inflammation.  (Stained  with  aniline  blue-black.) 
Distended  vessels  are  all  surrounded  by  thick  zones  of  clear  tissue,  in  places 
divided  obliquely,  while  outside  this,  ramifying  tracts  of  darker  tissue  extend  into 
the  white  substance.  In  D,  from  the  front  of  the  posterior  column,  tlie  changes 
are  shown  more  highly  magnified.  The  zone  around  the  vessels  is  seen  to  consist 
of  the  perivascular  sheath  distended  by  cellular  elements,  round,  oval,  fusiform, 
angular.  The  v/hite  substance  is  crammed  with  similar  cells,  and  hardly  any 
trace  of  nerve-fibres  or  of  normal  structure  can  be  perceived.  In  E,  from  a  less 
affected  part  of  the  lateral  column,  stained  with  carmine,  the  alveoli  remain, 
although  the  tissue  between  them  is  thickened,  and  in  many  places  studded  with 
refracting  grranules  and  larger  nuclei.  In  many  spaces  nerve-fibres  are  seen 
much  smaller  than  normal  in  consequence  of  the  wasting  of  the  white  substance. 
P  represents  a  similar  condition,  with  fewer  nerve-fibres,  and  extensive  infiltration 
of  the  alveoli  with  blood-corpuscles.  (From  sections  prepared  by  Dr.  Monej.  For 
the  specimen  I  am  indebted  to  the  late  Dr.  Haddeu.) 


356  SPINAL    CORD. 

pyramidal  tracts,  so  that,  an  inclx  or  two  above  the  U2)per  limit  of  the 
general  inflammatory  changes,  an  ascending  inflammation  of  these 
tracts  may  be  combined  with  an  ascending  degeneration  of  the  tracts 
that  always  present  secondary  ascending  degeneration. 

If  the  patient  survives,  the  new  interstitial  tissue  slowly  undergoes 
changes  which  give  it  a  fibroid  aspect,  although  for  some  time  cell- 
forms  may  preponderate  in  it.  We  know  very  little  of  the  nature  of 
the  process  that  occurs  in  the  cases  which  slowly  recover,  and  sub- 
serves restoration  of  function.  The  fact  that  a  long  period  of  total 
palsy  may  be  succeeded  by  the  slow  return  of  considerable  power, 
shows  that  even  g]-eatly  damaged  fibres  may  regain  functional 
capacity.  It  is  easy  to  conceive  that  the  fibres  which  are  only  so  far 
changed  that  the  axis-cylinders  are  swollen,  and  the  myelin  stains 
readily,  may  speedily  recover.  We  can  also  understand  that  the 
fibres  in  which  a  very  narrow  layer  of  myelin  remains  around  the 
axis-cylinder  may  also  regain  the  power  of  conducting.  We  must 
presume  that  in  these  fibres  there  is  no  interruption  of  the  axis- 
cylinders,  but  there  are  cases  in  which  we  can  scarcely  believe  that 
the  axis -cylinders  retain  their  continuity,  although  conducting  capacity 
is  ultimately  restored.  An  absolute  motor  palsy  of  twelve  months' 
duration  must  depend  on  changes  that  involve  an  absolute  inter- 
ruption of  the  fibres,  and  yet  in  such  a  case  some  return  of  power 
may  occur.  Is  this  e:ffected  by  a  growth  of  new  fibres  such  as  occurs 
in  nerves  ?  In  the  lower  animals  such  a  growth  of  fibres  has  been 
proved  to  occur ;  in  man  it  has  not  yet  been  demonstrated,  but  it  is 
difficult  to  conceive  any  other  explanation  of  the  clinical  facts.  The 
very  interesting  appearance  presented  by  the  section  of  a  cord  shown 
in  Figs.  103,  b,  and  104  suggests  strongly  a  process  of  regeneration. 
The  cord  was  crushed  in  the  mid- dorsal  region  by  a  fracture  of  the 
spine,  and  motor  and  sensory  paralysis  remained  absolute,  up  to  the 
level  of  the  lesion,  till  the  patient's  death,  six  months  later.  At  the 
seventh  cervical  (Fig.  103,  a)  the  cord  presents  only  the  usual  as- 
cending degenerations,  but  at  the  eighth  cervical  (Fig.  103,  b)  opaque 
tracts  extend  through  the  grey  and  white  matter,  and  adjacent  to 
these  there  is  everywhere  an  extensive  destruction  of  nerve-fibres.  In 
these  tracts  within  the  lateral  columns  a  large  number  of  very  minute 
nerve-fibres  can  be  seen,  each  consisting  of  a  fine  axis-cyhnder  sur- 
rounded by  myelin  (Fig.  104,  c).  The  fibres  are  smaller  than  any 
met  with  in  the  normal  cord.  The  appearance  is  as  if  there  had 
been  an  irregular  ascending  myelitis,  which  had  extended  up  the 
cord  as  high  as  between  the  seventh  and  eighth  cervical  segments, 
and  from  the  lower  extremity  of  the  normal  fibres  there  had  occurred 
a  growth  of  new  fibres  such  as  effects  the  regeneration  of  nerves.* 
We  seem  to  have  here  an  actual  process  of  renewal  of  fibres  that  had 
been  destroyed  by  such  inflammation  as  has  caused  the  empty  spaces 

*  For  these  sections  I  am  indebted  to  Dr.  F.  G.  Penrose.  Unfortunately  I  have 
been  unable  to  obtain  any  other  sectious  of  this  part  of  the  cord. 


ACUTE    MYELITIS. 


857 


Fig.  103. — AscendnigmvelUisfroTn  fr.ictureof  spine  completely  destroying  the  cord 
in  the  iniddorsal  region.  A,  from  the  seventh  cervical,  presents  only  ascending 
degenerations  in  post.  med.  col.,  direct  cerebel.  areas,  and  ant.-lat.  ascending  tract. 
B,  from  the  eighth  cervical,  presents  also  numerous  areas  of  finely  granuLir  aspect 
under  a  low  magnifying  power,  the  finer  structure  of  which  is  shown  in  Fig.  104. 


km^&A&:'. 


I 


€ 


Fig.  104. — From  the  outer  part  of  the  front  of  the  lateral  column  in  B,  Fig.  103. 
a  is  the  anteru-latcral  ascending  tract,  the  fibres  completely  degenerateil,  except 
a  few,  probably  of  other  nature  and  belonging  to  h,  a  narrow  zone,  nearly  healthy, 
of  the  antero-lateral  ground  fibres,  c  represents  the  outer  part  of  one  of  the 
finely  grannlar  tracts.  Under  the  higher  magnifying  power  these  tracts  are  seen 
to  consist  of  very  minute  nerve-fibres  with  a  grannlar  material  between  them  in 
which  few  cell-forms  can  be  distinguished.  Adjacent  to  these  tracts  there  has 
apparently  been  extensive  destruction  of  the  fibres,  large  spaces  being  occupied 
by  products  of  degeneration  and  appearing  empty  in  the  cleared  section. 

in  the  vicinity.  If  tMs  is  a  correct  interpretation  of  the  appearances 
they  constitute  proof  that,  even  in  the  huraan  cord,  it  is  possible  to 
have  a  new  formation  of  destroyed  fibres.     'Ihat  pyramidal  fibres  may 


358  SPINAL    COED. 

be  renewed  after  tliey  liave  been  degenerated  in  consequence  of  pres- 
sure, there  is  abundant  clinical  evidence,  since  complete  paralysis  with 
an  intense  spastic  state  of  tbe  limbs  may  be  recovered  from  perfectly, 
even  tbough.  it  lias  lasted  for  many  montlis.  Such,  an  event  is 
common  in  cases  of  pressure-myelitis  {q.  v.),  and  a  remarkable  in- 
stance will  be  mentioned  in  the  account  of  tumours  of  the  cord.  But 
in  such  cases,  although  it  is  not  likely  that  the  axis-cylinders  of  the 
fibres  have  preserved  their  integrity,  the  neuroglial  space,  which 
represents  the  nerve-sheath,  has  probably  remained  intact,  and  the 
re- growth  of  a  fibre  within  it  is  a  far  simpler  thing  than  a  new  forma- 
tion of  fibres  in  an  area  in  which  all  the  morphological  framework 
has  perished. 

Symptoms. — The  symptoms  of  myelitis  vary  much  according  to  its 
degree  and  form.  The  most  common  variety,  which  may  be  regarded 
as  the  typical  form,  is  acute  transverse  myelitis,  and  to  this  especially 
the  following  description  applies.  The  conspicuous  symptoms  are 
those  which  depend  on  the  interference  with  the  functions  of  the  cord, 
and  these,  in  most  cases,  are  also  the  first  indications  of  the  disease. 
They  are  sometimes  preceded,  and  more  often  accompanied,  by  general 
symptoms,  such  as  attend  inflammation  of  other  internal  organs- 
malaise,  shivering,  headaclie,  depression,  loss  of  appetite,  pyrexia, — 
symptoms  which  may  attend  any  form  of  acute  myelitis,  or  may  be 
altogether  absent.  When  due  to  cold,  and  commencing  some  days 
after  exposure,  general  rheumatic  pains  may  occur  in  the  interval. 

The  spinal  symptoms,  if  not  prominent  at  first,  quickly  become  so. 
The  most  obtrusive  is  the  motor  weakness,  but  this  may  be  preceded, 
for  a  few  hours  or  days,  by  sensoiy  symptoms  in  the  limbs — "  numb- 
ness," tingHng,  or  burning  sensations.  Occasionally  there  are  rheu- 
matoid pains  at  the  onset,  sometimes  referred  chiefly  to  the  joints. 
Pain  may  be  felt  in  the  back  as  well  as  in  the  limbs,  but  the  verte- 
bral pain  is  seldom  prominent,  and  soon  ceases.  Occasionally  spas- 
modic twitchings  in  the  limbs  or  painful  cramps  attend  the  onset. 
In  rare  cases  there  is  a  general  convulsion.  This  is  most  frequent  in 
children,  and  then  may  be  sometimes  the  expression  of  the  general 
disturbance  of  the  system.  But  convulsion  occasionally  attends  the 
onset  of  acute  myelitis  in  adults,  even  when  there  is  no  cerebral 
complication.  Three  instances  of  this  have  come  under  my  own 
observation. 

The  motor  paralysis  usually  comes  on  rapidly,  and  reaches  a  con- 
siderable degree  in  the  course  of  a  few  hours.  A  patient  after  such 
sensations  as  have  been  described,  or  without  any  warning,  finds  that 
his  legs  feel  heavy  ;  after  walking  for  a  few  hours  he  is  obliged  to  sit 
down  and  rest.  When  he  tries  to  walk  again  his  legs  feel  "  as  if  made 
of  lead."  He  lies  down  for  an  hour,  and  then  finds  that  he  cannot 
stand,  and  in  a  few  hours  more  is  unable  to  raise  his  legs,  although 
perhaps  he  can  still  move  the  feet  or  toes  ;  next  day  even  this  power 
may  be  lost.     Occasionally  the  mode  of  onset  is  still  more  rapid,  and 


ACUTK    MYELITIS.  359 

occupies  only  a  few  minutes.  The  legs  are  found  suddenly  to  be 
heavy  and  tingling,  the  sufferer  sits  down  on  a  chair  for  a  quarter  of 
an  hour,  and  then  finds  that  he  cannot  stand.  Such  raj)id  onset 
resembles  that  of  spinal  haemorrhage,  and  it  is  probable  that  in  most 
of  these  cases  there  is  haemorrhage  in  addition  to  inflammation — 
"  hsemorrhagic  myelitis."  Such  cases  are,  however,  sufficiently  rare 
not  to  interfere  with  the  diagnostic  rule  that  a  sudden  onset  means  a 
vascular  lesion,*  and  that  the  characteristic  onset  of  myelitis  is  rapid 
but  not  sudden.  Now  and  then  the  onset  is  in  the  night,  during 
sleep ;  a  patient  goes  to  bed  well,  and  wakes  up  in  the  morning  with 
complete  paraplegia.  On  the  other  hand,  the  onset  of  the  paralysis 
may  occupy  several  days,  or  even  a  week;  when  longer  than  this, 
from  ten  to  forty  days,  the  myelitis  is  to  be  regarded  as  subacute. 
Occasionally  the  onset  is  bj  a  series  of  sudden  attacks  of  weakness  at 
intervals  of  a  few  hours  or  days,  or  even  weeks.  In  the  latter  case 
there  may  be  some  recovery  from  one  attack  before  the  next  comes  on. 
Frequently  there  is  gradual  partial  loss  of  power  for  a  few  days,  and 
then  complete  paralysis  comes  on  rapidly;  the  first  symptoms  may 
be  slight,  and  their  nature  only  recognised  when  the  disease  has 
developed. 

When  the  paralysis  has  reached  its  height,  and  has  ceased  to 
increase,  it  is  usually  complete ;  sometimes  it  is  incomplete,  the  limbs 
can  be  moved,  but  with  little  power,  or  the  patient  may  be  able  to 
contract  certain  muscles,  but  not  to  move  the  parts  to  which  they  are 
attached,  perhaps  only  to  move  the  toes.  The  distribution  of  the 
paralysis  depends  on  the  position  and  extent  of  the  disease.  In  the 
majority  of  cases,  the  legs  and  lower  part  of  the  trunk  only  are 
affected,  because  the  dorsal  region  of  the  cord  is  the  most  frequent 
seat  of  myelitis.  If  it  is  incomplete  in  the  legs,  the  flexor  muscles 
usually  suffer  more  than  the  extensors,  although  this  may  not  be  appa- 
rent as  the  patient  lies  in  bed,  because  the  leverage  at  the  knee  renders 
necessary  considerable  power  to  extend  the  joint  against  gravitation. 
When  the  disease  is  in  the  cervical  region  the  arms  as  well  as  the  legs 
are  involved,  and  the  arms  are  sometimes  paralysed  before  the  legs. 
If  the  lesion  is  extensive  in  the  cervical  region,  the  damage  to  the  grey 
matter  and  root-fibres  causes  atrophic  palsy  in  the  arms,  while  the 
legs  are  the  seat  of  simple  and  tdtimately  spastic  paralysis.  In  such 
cases  the  intercostals  are  paralysed,  and  breathing  is  carried  on  by 
the  diaphragm.  Myelitis  above  this  level,  if  complete,  abolishes  all 
respiratory  power,  and  necessarily  causes  death  with  great  rapidity; 
but  occasionally,  in  partial  inflammation,  the  diaphragm  may  escape, 
or  the  intercostals  retain  some  power  and  life  be  preserved.  In  such 
rare  cases  the  arms  as  well  as  the  legs  may  be  the  seat  of  simple 
palsy,  and  the  neck  muscles  may  become  atrophied.  This  was  seen 
in  one  patient,  in  whom  the  sterno-mastoids  and  the  upper  part  of 

•  Indeed,  it  is  possible  that  these  cases  constitute  no  exception  to  the  rule,  the 
mje^'^iia  being  set  up  by  vascular  obstruction. 


360  SPINAL    CORD. 

eacli  trapezius  were  wasted,  while  tlie  arms  as  well  as  the  leg& 
presented  an  increase  of  myotatic  irritability.  In  such  cases  dis- 
seminated inflammation  may  spread  into  the  medulla,  the  pharynx 
and  the  tongue  may  be  affected,  vomiting  may  occur,  and  the  heart's 
action  be  interfered  with.  The  range  of  motor  symptoms,  when  the 
disease  is  at  different  heights,  is  indicated  in  the  table  of  functions 
(p.  •Z5'2)._ 

Sensation  is  frequently  impaired  as  well  as  motion.  At  the  onset, 
indeed,  it  is  involved  in  some  degree  in  all  cases  of  general  transverse 
myelitis.  In  severe  cases  it  is  absolutely  lost  up  to  the  level  of  the 
lesion.  The  upper  limit  of  loss  is  often  oblique,  being  higher  in  front 
than  behind.  In  slighter  cases  the  loss  may  be  partial,  and  only 
certain  forms  of  sensation  may  be  impaired,  either  in  consequence  of 
the  position  of  the  disease,  when  touch  or  pain  may  be  lost  alone,  or 
of  its  slight  degree,  when  touch  is  generally  impaired  and  painful 
sensibility  preserved.  Occasionally  there  is  general  hyiDersesthesia  in 
the  limbs  at  the  onset.  At  the  level  of  the  lesion  there  is  usually  a 
zone  of  hypersesthesia,  corresponding  to  the  distribution  of  the  nerves 
that  pass  through  the  upper  part  of  the  affected  region,  i.  e.  through 
the  lowest,  merely  irritated,  portion  of  the  cord  above  the  disease. 
The  hypersesthesia  may  readily  be  detected  by  passing  a  hot  sponge 
down  the  spine ;  the  sense  of  warmth  changes,  at  the  hypersesthetic 
zone,  into  one  of  pain.  Corresponding  to  this  zone  of  hyperaesthesia 
there  is  often  a  sense  of  painful  constriction,  a  "girdle  pain,"  which 
may  persist  for  a  long  time,  even  after  the  loss  of  sensibility  below 
the  lesion  has  passed  away.  Occasionally  it  only  comes  on  some  time 
after  the  onset,  probably  from  cicatricial  compression  and  irritation  of 
the  root-fibres.  Its  position  varies  with  that  of  the  disease.  It  is 
most  commonly  felt  between  the  umbilicus  and  ensiform  cartilage, 
Bometimes  around  the  lower  part  of  the  abdomen,  about  the  anus,  or 
in  the  legs.  It  is  of  much  practical  importance  because  it  is  evidence 
of  the  existence  of  organic  disease,  and  also  of  the  upper  limit  of  the 
lesion.  It  is  sometimes  a  very  early  symptom.  Any  initial  pain  in 
the  limbs  usually  ceases  when  sensation  becomes  abolished.  When 
pain  persists  in  the  legs,  it  is  often  less  when  these  are  flexed. 

The  state  of  reflex  action  varies,  and  depends  on  the  position  of  the 
disease,  in  accordance  with  the  laws  already  stated  (p.  262).  An 
acute  lesion  in  any  part  of  the  cord  may  cause  an  initial  inhibitory 
loss  of  reflex  action  in  the  parts  below ;  but  if  the  lesion  is  above  the 
lumbar  enlargement,  reflex  action  returns  in  the  course  of  a  few 
hours.  Frequently  there  is  no  initial  depression.  Subsequently  the 
reflex  action  becomes  excessive,  that  from  the  skin  rapidly,  that  from 
the  muscles  more  slowly„  Ultimately  each  attains  a  high  degree  of 
exaltation.  If  the  disease  involves  the  lumbar  enlargement  all  forms  • 
of  reflex  action  are  lost.*  When  in  the  cervical  region  it  may  be  lost 
in  the  arms,  excessive  in  the  legs.  In  dorsal  myelitis  the  trunk- 
*  See,  however,  the  facts  stated  on  p.  264. 


ACUTE    MYELITIS.  361 

reflexes  are  often  impaired,  and  may  give  important  information  as  to 
the  seat  of  the  disease.  The  abdominal  reflexes  should  be  examined 
at  various  levels,  and  the  greater  impairment  of  these  on  one  side 
than  on  the  other  may  reveal  clearly  the  unequal  distribution  of  the 
lesion  and  its  vertical  extent. 

The  muscles  of  the  legs  are  sometimes  at  first  flabby  and  toneless 
during  the  stage  of  initial  depression  of  reflex  action,  doubtless  from 
the  same  influence.  Ttds  condition  soon  passes  off  if  the  lesion  is 
above  the  lumbar  enlargement,  and  as  reflex  action  returns  the 
muscles  regain  their  tone.  If,  however,  the  myelitis  involves  the 
lumbar  enlargement  in  considerable  degree,  the  muscles  of  the  legs 
remain  flaccid,  and  lose  all  influence  on  the  posture  of  the  limbs. 
The  feet  fall  into  an  extended  position,  so  that  the  instep  is  in  a  line 
with  the  tibia  (Fig.  105).    The  muscles  also  waste  rapidly,  and  often 


/ 


Fia.  105. — Posture  of  feet  in  myelitis  of  lumbar  enlargement  with  rapid 
wasting  of  muscles. 

present  the  reaction  of  degeneration.  The  wasting  in  these  cases  is 
often  extreme.  In  many  cases  of  myelitis  above  the  lumbar  enlarge- 
ment there  is  a  slighter  and  slower  wasting  of  the  muscles,  without 
loss  of  reflex  action,  and  generally  with  excess  of  the  muscle-reflexes ; 
the  ultimate  amount  of  wasting  may  be  considerable,  but  it  is  never 
so  great  as  when  the  lumbar  grey  matter  is  diseased.  There  is  never 
the  reaction  of  degeneration,  but  only  a  slight  change  in  irritability, 
similar  to  each  current ;  first  an  increase,  which  after  some  weeks  or 
months  gives  place  to  a  slight  diminution. 

The  sphincters  are  usually  affected  from  the  first,  except  in  very 
slight  cases,  and  they  often  afford  the  earliest  indication  of  the  com- 
mencing lesion.  There  is  usually  first  retention  of  urine,  which  not 
rarely  exists  for  several  hours,  or  even  for  a  day  or  two,  before  other 
symptoms  are  added  to  it.  There  is  afterwards  incontinence,  over- 
flow or  reflex  (see  p.  247),  when  the  disease  is  above  the  lumbar 
region;  if  the  lumbar  centre  is  diseased  there  is  persistent  incon- 
tinence from  the  first  without  retention.  In  the  former  case  there  is 
involuntary  action,  in  the  latter  inaction,  of  the  sphincter  ani,  and  in 
both  cases  incontinence  of  faeces.  This  may  be  inconspicuous  in  the 
early  period,  on  account  of  constipation,  due  to  intestinal  paralysis. 
In  cases  of  partial  myelitis,  as  of  the  anterior  cornua,  the  siDhincters 
are  often  unaffected.     The  urine  is  frequently  alkaline  in  reaction. 


302  SPINAL    COED. 

and  the  change  sonietinios  comes  on  so  rapidly  that  it  is  evidently 
the  result  of  an  alteration  in  the  secretion.  In  all  cases  of  retention, 
decomposition  in  the  bladder  increases  the  alkalinity.  Cystitis  often 
follows,  so  that  the  urine  contains  pus. 

The  temperature  of  the  paralysed  limbs  is  usually  at  first  raised  one 
or  two  degrees  abore  that  of  the  mouth,  but  it  afterwards  falls,  and 
remains  a  degree  or  so  lower  than  that  elsewhere.  The  skin  is  often 
dry,  sometimes  covered  with  sweat.  Its  nutrition  often  suffers,  and 
bedsores  result.  In  some  cases  they  are  due  to  long-continued  pres- 
sure and  neglect,  but  they  may  occur  early  and  intensely  when  the 
disease  affects  the  lumbar  enlargement  or  irritation  extends  down  to 
it  from  above.  The  least  pressure  on  the  skin  may  then  cause  a 
bulla  containing  sanious  liquid,  and  sloughing  occurs  vsdth  readiness, 
especially  over  the  sacrum.  Now  and  then  there  is  effusion  into  the 
knee-joints.  Occasionally  the  tendency  to  sloughing  of  the  sldn  is  so 
intense  that  the  lesions  appear  to  be  spontaneous. 

Such  trophic  disturbances  sometimes  occur  in  the  cellular  tissue  of 
the  lower  part  of  the  body,  especially  in  the  neighbourhood  of  the 
bladder  and  rectum.  A  x-emarkable  example  of  such  disturbance 
was  presented  by  the  case  from  which  Figs.  98  and  99  are  taken. 
Symptoms  of  subacute  myelitis  were  followed  by  those  of  cellulitis  in 
the  lower  part  of  the  abdomen,  on  account  of  which  the  patient  was 
admitted  into  University  CoUege  Hospital  under  the  care  of  my  col- 
league, Mr.  Marcus  Beck.  Subcutaneous  emphysema  developed  about 
the  groins,  and  suppuration  occurred  near  the  rectum.  In  conse- 
quence of  this  inflammation  the  patient  died,  the  cord  symptoms 
having  slowly  increased ;  after  death  no  lesion  of  the  intestine  or  local 
cause  of  the  cellulitis  could  be  discovered.  It  is  probable  that  the 
derangement  of  the  trophic  influence  of  the  cord  facilitates  the  occur- 
rence of  cystitis  from  retention  of  urine,  and  intensifies  its  effect  on 
the  kidneys.  Ulceration  of  the  bladder,  which  sometimes  occurs  with 
remarkable  rapidity,  may  be  thus  produced,  or  a  small  abscess  may 
form  in  the  wall  of  the  bladder,  and  thus  an  opening  may  be  formed 
into  the  peritoneal  cavity,  and  fatal  peritonitis  may  be  induced.  A 
vesico-vaginal  fistula  may  be  developed  in  the  same  way.  Ulceration 
of  the  urethra,  and  consequent  extravasation  of  urine,  have  been 
observed  under  similar  conditions.*  In  another  case  the  inflamma- 
tion of  the  bladder  was  intense,  and  suppurative  cellulitis  occurred 
outside  the  organ  and  set  up  a  fatal  peritonitis ;  the  kidneys  were  the 
seat  of  very  acute  suppurative  inflammation.f  These  effects  of  cystitis 
may  probably  always  be  prevented. 

When  the  disease  is  in  the  cervical  region  the  pupil  may  be  affected. 
In  rare  cases  of  myelitis,  optic  neuritis  has  been  observed,  without 
any  intra-cranial  complication  to  cause  it.|     It  is  probably  not  the 

*  S.  West,  'St.  Bartli.  Hosp.  Rep.,'  vol.  x. 

t  Sharkey  and  Lawford,  '  Trans.  Ophtli.  Soc.,'  1883. 

J  Optic  neuritis  was  present  in  the  case  of  disseminati  d  myelitis  under  the  care 


ACUTE    MYELITIS.  363 

resiilt  of  the  inflammation  of  the  spinal  cord,  but  is  an  associated  and 
similar  lesion,  the  result  of  the  cause  of  the  myelitis,  which,  in  such 
cases,  is  probably  a  blood-state.  It  is  noteworthy  that  most  of  the 
cases  thus  accompanied  have  been  instances  of  disseminated  myelitis, 
a  form  that  suggests  a  cause  acting  widely  on  the  nervous  system. 
In  one  case  *  the  optic  neuritis  reached  its  height  some  weeks  before 
the  occurrence  of  the  first  spinal  symptoms,  and  in  the  cord  were  two 
separate  and  distant  foci  of  inflammation;  in  another  the  neuritis 
was  found  a  fortnight  before  the  onset  of  disseminated  myelitis. f 

In  cases  of  rapid  development  the  degree  attained  by  the  symptoms 
at  their  onset  may  not  be  exceeded.  In  some  cases  a  slower  increase 
or  extension  ensues  in  the  course  of  a  few  days.  The  myelitis,  begin- 
ning below,  may  ascend  the  cord,  and  the  arms  and  respiratory 
muscles  may  gradually  become  involved.  The  upward  extension  may 
be  steadily  progressive,  or  it  may  occur  in  successive  separate  attacks. 
In  the  latter  case,  it  seldom  extends  above  the  dorsal  region.  Or 
inflammation,  beginning  in  the  dorsal  region,  may  slowly  descend  into 
the  lumbar  enlargement.  The  extension  may  be  by  the  grey  matter, 
especially  of  the  anterior  cornua,  or  by  the  posterior  region.  In  each 
case  the  extension  downwards  is  indicated  by  loss  of  the  reflex  action 
present  in  the  earlier  stage :  in  the  one  there  is  rapid  muscular  wasting 
with  loss  of  faradic  irritability  ;  in  the  other  the  muscles  retain  their 
nutrition  and  irritability,  but  sensation,  if  before  present,  becomes 
lost. 

The  constitutional  symptoms,  which  have  been  mentioned  as  attend- 
ing the  onset,  vary  greatly  in  their  severity  and  course.  They  are,  as 
a  rule,  less  in  the  simple  transverse  myelitis  than  in  the  disseminated 
form.  In  most  cases  they  reach  their  height  on  the  second  or  third 
day  after  the  onset  of  severe  symptoms.  Thus  in  one  case  the  tempe- 
rature was  normal  on  the  first  day  of  indisposition,  when  the  patient 
complained  only  of  slight  headache  and  slight  numbness  of  the  legs. 
During  the  next  three  days  these  gradually  became  completely  para- 
lysed, and  the  temperature  was  successively  102°,  103°,  and  104'5'*, 
and  then  gradually  fell  to  normal  at  the  end  of  a  week.  In  ^mother 
case,  in  which  the  toxaemic  state  was  part  of  that  of  measles,  the  tem- 
pei-ature  rose  from  100°  to  103-8°  on  the  first  day  of  the  symptoms  of 
myelitis,  and  on  the  next  day  to  104*2°,  but  fell  (partly  from  anti- 
pyrin)  on  the  third  day,  on  wliich  death  occui-red,  to  100-2°.J  In  less 
acute  cases,  in  which  foci  of  inflammation  develop  progressively  with 
spreading  palsy,  during  one  or  two  weeks,  moderate  irregular  pyrexia, 
99°  to  101°,  may  go  on  as  long  as  the  disease  is  increasing,  and  may  be 
renewed  at  any  subsequent  relapse.    The  temperature  is  also  raised 

of  Dr.  Dresclifeld  from  which  Fig.  100  is  taken,  and  also  in  that  of  Sharkey  and 
Lawtord  just  mentioned.     See  '  Medical  Ophthalmoscopy,'  3rd  ed.,  p.  ISd* 

*  Sharkey  and  Lawford,  op.  cit. 

t  Achard  and  Quinon,  '  Arch,  de  Med.  exp.,'  1889. 

j  Barlow,  'Med.-Chir.  Trans.,'  1887,  p.  76. 


864  SPINAL  COED. 

by  any  complications,  and  especially  "by  cystitis.  On  tlie  other  hand, 
in  simple  cases,  with  a  single  focus  of  inflammation,  the  initial  eleva- 
tion may  be  trifling,  and  after  the  first  day  or  two  the  temperature 
remains  normal. 

If  the  patient  survives,  the  course  of  the  symptoms  varies  according 
to  the  intensity  of  the  lesion.  The  paralysis,  motor  and  sensory,  may 
remain  complete.  More  frequently  sensation  is  recovered  after  a  few 
weeks  or  months,  .while  motor  power  continues  absent  for  a  much 
longer  period.  The  excessive  reflex  action  may  lead  to  the  gradual 
development  of  spasm  in  the  legs.  This  is  especially  related  to  the 
increase  of  the  muscle-reflex  action  which  follows  the  secondary  de- 
scending degeneration  of  the  pyramidal  fibres.  Ultimately  the  condi- 
tion is  that  termed  "  spastic  paraplegia,"  and  described  in  detail  in 
another  section.  The  occurrence  of  this  spasm  is  not  incompatible 
with  the  recovery  of  some  voluntary  power.  The  muscles  in  these  cases 
are  often  well  nourished,  and  may  even  increase  in  size ;  the  spasm 
constitutes  a  powerful  stimulus  to  their  growth.  Spasm  may,  however, 
co-exist  with  the  slow  moderate  wasting  described  above  (p.  265),  but 
is  rarely  intense  in  these  cases. 

In  some  cases,  flexor  spasm,  after  a  time,  tates  the  place  of  bhe 
extensor  spasm,  at  first  in  transient  attacks  but  at  last  permanently, 
and  becomes  fixed  by  contracture  and  shortening  of  the  flexor 
muscles.  It  may  be  so  great  that  the  knees  are  against  the  abdo- 
men and  the  heels  against  the  nates.  This  substitution  of  flexor  for 
extensor  spasm  occurs  chiefly  in  cases  in  which  the  paralysis  is 
absohite,  and  the  organic  disease  of  the  cord  so  considerable  and  of 
such  duration  as  to  make  recovery  impossible.  It  is  therefore  a  very 
grave  sign. 

Death  may  occur  early  in  the  disease  from  respiratory  paralysis,  or 
from  the  extension  of  disseminated  myelitis  into  the  medulla  oblon- 
gata; subsequently  it  may  be  due  to  various  effects  of  the  trophic 
disturbance,  acute  or  chronic,  and  to  blood-states  induced  by  bed- 
sores or  by  kidney  disease  resulting  from  cystitis,  &c.,  mechanisms 
that  hpiVe  been  already  mentioned  in  the  account  of  the  symptoms. 
Improvement,  when  it  occurs,  is  usually  slow,  but  continues  for  a 
long  time,  and  recovery  may  be  complete  in  cases  of  moderate 
severity.  When  there  is  much  damage  to  the  cord,  however, 
recovery  is  often  imperfect,  and  some  weakness  remains,  accom- 
panied either  by  spasm  or  by  wasting.  It  is  rarely  that  no  improvement 
occurs ;  occasionally,  however,  the  palsy  remains  absolute,  although 
life  is  prolonged  for  years.  In  some  cases  improvement  occurs,  slight 
or  considerable,  and  is  followed  by  a  relapse,  which  leaves  the  patient 
worse  than  the  first  attack  ;  and  this  may  occur  again  and  again,  each 
relapse  being  the  expression  of  a  fresh  extension  of  the  inflammation 
in  the  cord.  This  relapsing  course  is  met  with  especially  in  the 
disseminated  form,  and  in  the  subjects  of  gout,  either  acquired  or 
inherited.     Other  cases,  again,  present  what  may  be   termed  a  re- 


ACUTE   MTEriTIS.  865 

current  course,  improvement,  and  perhaps  a  considerable  degree  of 
recovery,  continuing  for  months,  when  a  fresh  attack  occurs.  On 
the  other  hand,  in  many  cases,  especially  of  simple  transverse 
myehtis,  when  improvement  is  established  it  goes  on,  and  there  is  no 
tendency  to  recurrence. 

Varieties. — The  different  pathological  forms  of  myelitis  are  attended 
by  some  differences  in  the  symptoms.  Those  of  transverse  myelitis 
have  been  described  as  the  most  common  and  typical  manifestations 
of  the  disease.  Focal  myelitis,  in  which  there  is  a  single  spot  of 
inflammation,  not  extending  across  the  cord,  may  occur  in  various  parts 
of  the  cord ;  but,  on  account  of  the  slight  degree  of  severity  of  the 
symptoms,  little  is  known  of  them  except  when  the  focus  of  inflam- 
mation is  of  some  size  or  is  situated  in  one  anterior  cornu  (as  a 
variety  of  polio-myelitis),  because  such  cases  are  rarely  fatal.  It  is, 
however,  probable  that  this  variety  is  not  uncommon.  Cases  are 
occasionally  met  with  in  which  symptoms  of  very  limited  range  come 
on  acutely,  and  may  reasonably  be  ascribed  to  such  a  lesion.  They 
may  be  one-sided,  and  vai-ious  in  character,  often  beiag  limited  to  a 
single  limb. 

A  much  more  frequent  focal  form  is  disseminated  myelitis,  in  which 
there  are  several  foci  of  inflammation  in  the  same  or  different  parts 
of  the  cord.  The  onset  of  this  form  iB  often  subacute,  and  constitu- 
tional symptoms  are  frequently  absent.  The  most  important  special 
symptoms  are  those  which  indicate  interference  with  the  central  func- 
tions of  the  cord  in  more  than  one  locality,  but  the  combinations 
produced  are  very  variable.  In  many  cases  the  several  foci  of  in- 
flammation develop  successively,  not  simultaneously,  and  then  we  have 
a  corresponding  succession  of  symptoms.  Thus  a  myelitis  in  the 
cervical  region,  causing  atrophy  of  some  groups  of  muscles  in  one  arm 
and  paralysis  of  the  corresponding  leg,  may  be  followed  by  paralysis 
of  the  other  leg  with  such  loss  of  reflex  action  in  it  as  shows  a 
separate  focus  of  inflammation  in  the  lumbar  enlargement,  and  this 
again  by  the  development  of  a  gu'dle  pain  corresponding  to  the 
middle  of  the  dorsal  region,  from  a  fresh  area  of  inflammation  in 
that  situation.  When  numerous  foci  of  myelitis  occur  in  the  dorsal 
region,  as  is  not  uncommon  in  syphilitic  cases  and  after  injury,  the 
symptoms  may  closely  resemble  those  of  a  transverse  myelitis,  because 
by  one  or  another  of  these  foci  each  of  the  elements  of  the  cord  is 
interrupted.  The  only  distinction  may  be  the  extensive  impairment 
of  the  trunk-reflexes,  or  the  detection  of  loss  of  irritability  in  the 
corresponding  muscles.  Closely  allied  to  disseminated  myelitis  is 
the  condition  known  as  disseminated  encephalomyelitis,  described  by 
Leyden  over  twenty  years  ago  under  the  name  of  acute  ataxy.  It  is 
characterised  by  the  presence  of  small  foci  of  change  in  the  cord,  pons, 
and  cerebrum.  In  some  cases  such  foci  have  only  been  found  in  the 
cord.  The  symptoms  are  those  of  interference  with  the  functions  of 
the  parts  in  which  the  foci  are  formed,  e.g.  defects  of  speech  and 


366  SPINAL    COED. 

articniatlon,  tremor  and  ataxy  in  the  legs  and  arms,  tremor  of  the 
head,  and  ejcaggerated  reflexes  associated  with  a  spastic  gait.  The 
condition  seems  to  be  frequently  dependent  upon  some  antecedent 
infectious  disease  or  other  toxic  blood-state,  e.  g.  inhalation  of  a 
poisonous  gas.  It  is  more  frequent  in  children,  is  variable  in  its 
course,  sometimes  followed  by  complete  and  fairly  rapid  recovery,  in 
other  cases  running  a  more  chronic  course  with  altematiug  relapse 
and  recovery.  Sometimes  it  leads  to  permanent  disablement  or  im- 
pairment of  motor  functions.  The  sphincters  may  escape,  and  the 
mental  condition  is  not  as  a  rule  impaired,  except  perhaps  at  the  onset, 
which  may  be  characterised  by  unconsciousness  and  delirium.  Ac- 
cording to  some  observers,  the  condition  may  merge  into  one  not  dis- 
tinguishable from  disseminated  sclerosis.  Leyden,  however,  asserted 
that  disseminated  encephalomyelitis  has  no  tendency  to  extend. 

In  the  rare  form  of  diffuse  central  myelilis  there  is  usually  rapid 
loss  of  power,  of  sensation,  and  of  reflex  action,  considerable  elevation 
of  temperature,  speedy  trophic  disturbance,  and  often  death  at  the 
end  of  two  or  three  days.  Rarely  sensation  and  reflex  action  have 
been  lowered  and  not  lost.  On  the  other  hand,  the  loss  of  sensation 
is  said  to  be  sometimes  absolute,  when  motor  palsy  is  incomplete. 
The  symptoms  have  begun  in  arms  and  legs  simultaneously,  or  in 
either  of  these,  and  have  accordingly  spread  upwards  or  downwards. 
Practically  nothing  is  yet  known  of  any  slight  non-fatal  forms  of  this 
variety.  Hssmorrhagic  myelitis  is  scarcely  a  special  form,  since  any 
acute  inflammation  of  the  cord  may  be  attended  by  a  sudden  extrava- 
sation of  blood.  Its  manifestation  is  the  sudden  onset  of  severe 
symptoms  after  slighter  disturbance,  such  as  indicates  a  commencing 
myelitis. 

Parenchymatous  myelitis  is  fcaown  only  in  a  few  forms,  and  we  have 
much  yet  to  learn  regarding  it.  In  diphtheritic  paralysis  there  is  an 
acute  degenerative  change  in  the  nerve-elements,  especially  in  the 
nerve-cells,  which  must  be  regarded  as  essentially  a  lesion  of  this 
form.  In  some  cases  of  polio-myelitis  the  motor  nerve-cells  suffer 
primarily,  and  the  interstitial  tissue  is  not  affected  except  in  the 
severer  cases  of  this  form.  In  all  inflammations,  whether  parenchy- 
matous or  interstitial  in  nature,  all  the  tissues  of  an  organ  tend  to 
be  involved  if  the  process  is  acute.  Other  symptoms  that  are  of  im- 
portance, as  suggesting  this  variety,  are  those  of  acute  ataxy,  clearly 
dependent  on  an  affection  of  the  cord  occasionally  met  with.  Thus 
a  married  woman,  who  was  certainly  the  subject  of  syphilis,  and 
had  presented  secondary  symptoms  a  year  previously,  found  one 
day  that  she  could  not  walk  so  weU  as  usual,  and  the  next  morning 
could  not  stand,  on  account  of  extreme  inco-ordination  in  the  legs. 
When  she  tried  to  use  them,  the  resemblance  to  the  extreme  degree 
of  locomotor  ataxy  was  perfect.  The  knee-jerk  was  normal  on  the 
right  side,  almost  lost  on  the  left.  The  left  leg  was  thought  to  be  a 
little  weak,  but  in  a  day  or  two  its  power  was  good.    For  the  first 


ACUTE    MYELITIS.  307 

few  days  there  "was  great  liyperaestliesia  of  tlie  lowei  part  of  the 
trunk  and  legs,  but  at  the  end  of  a  week  this  was  reduced  to  a  band 
at  the  level  of  the  lower  haK  of  the  abdomen,  where  even  a  touch 
occasioned  pain  ;  subsequently  a  girdle  pain  developed  in  this  region. 
Her  condition  lasted  unchanged  for  two  months,  and  then,  iodide  and 
belladonna  being  given,  slowly  improved ;  but  it  was  not  until  four 
months  after  the  onset  that  she  could  walk  a  little,  and  then  only  in 
a  highly  ataxic  manner.     Her  recovery  was  ultimately  perfect. 

In  another  group  of  cases,  of  which  I  have  seen  several  instances, 
inco- ordination  comes  on  acutely  in  one  arm,  and  may  be  accompanied 
by  complete  muscular  anaesthesia,  so  that  the  power  of  estimating 
weights  is  absolutely  lost,  a  poker  and  feather  seeming  alike,  although 
cutaneous  sensibility  is  perfect.  The  condition  has  reached  its  height 
in  the  course  of  a  few  hours,  remained  complete  for  weeks,  and  slowly 
passed  away,  in  the  cases  I  have  seen.  The  precise  nature  is  uncertain, 
but  provisionally  it  seems  best  to  class  them  together  as  cases  of  acute 
myelitic  ataxy.*  Their  dependence  on  an  organic  spinal  lesion  seems 
certain,  and  the  onset  of  this  is  incompatible  with  any  other  than  an 
inflammatory  process.  It  is  possible  that  some  of  these  cases  are  due 
to  a  focus  of  ordinary  inflammation,  so  situated  as  to  affect  structures 
of  a  common  function,  while  others  may  be  parenchymatous. 

Pathology. — Yery  little  is  known  of  the  pathology  of  acute 
myelitis, — that  is,  of  the  actual  mechanism  by  which  it  is  produced, 
of  the  nature  of  the  causes  to  which  it  is  due,  and  of  the  relation  of 
the  morbid  process  to  its  causes.  The  spinal  cord  differs  from  most 
organs  in  its  great  liability  to  primary  inflammation,  in  which  it 
presents  a  remarkable  contrast  to  the  brain.  The  liability  is  not 
equally  distributed  in  the  cord,  but  is  least  in  the  parts  nearer  the 
brain,  less  in  the  cervical  than  the  lumbar  enlargement,  and  less  in 
these  than  in  the  dorsal  region,  so  far  as  the  most  common  form  is 
concerned.  The  special  liability  of  the  dorsal  region  to  transverse 
myelitis  may  be  associated  with  the  readiness  with  which  this  part 
undergoes  post-mortem  softening,  but  we  are  ignorant  of  the  condi- 
tions by  which  this  liability  is  determined.  The  disseminated  form 
has  less  tendency  to  be  localised  in  any  special  part.  A  question 
of  considerable  interest  is  whether  acute  inflammation  of  the  cord 
always  begins  as  such,  or  whether,  in  any  case  or  form,  it  is  set  up 
by  vascular  obstruction,  such  as  thrombosis  in  a  minute  vessel.  It 
is  conceivable  that  such  an  initial  lesion  may  ultimately  disappear 
in  the  intense  inflammation  it  excites. f  Myelitis  may  be  readily 
produced  by  the  arrest  of  the  blood-supply  even  for  a  short  time,  as 
numerous  experimenters  have  shown,  but  their  researches  throw  httle 
light  on  the  disease  as  it  occurs  in  man ;  the  effect  is  to  produce  first 

*  For  a  typical  case  apparently  of  this  character  see  Campbell  Thomson, 
'  Lancet,'  1897,  vol.  ii,  p.  1586. 

+  Williamson  ('  Lancet,'  1894)  has  shown  that  myelitis  is  sometimes  the  result 
of  obstruction  occurring  in  yessels  with  syphilitic  endarteritis. 


368  SPINAL  COED. 

necrotic  breaking  up  of  the  nerve-elements,  and  long  after  this  the 
vascular  lesions  of  ordinary  interstitial  myelitis.  The  processes  are 
essentially  different.  But  the  experiments  show  that  the  lumbar 
enlargement  is  thus  damaged  with  special  readiness,  and  this  suggests 
that  the  proclivity  to  disease  of  this  part  is  due  to  its  inherent  sus- 
ceptibility. 

At  present  the  questions  of  chief  interest  are  the  extent  to  which 
the  disease  is  due  to  a  morbid  blood-state,  the  probable  nature  of  such 
a  cause,  and  the  mechanism  by  which  it  acts.  The  chief  facts  that 
throw  light  on  the  subject  have  been  mentioned  already,  the  most 
important  being  the  occurrence  of  myelitis  in  the  acute  specific 
diseases  (in  which  we  must  ascribe  it  to  the  organised  virus  of  the 
primary  malady  itself,  to  some  secondary  but  associated  organised 
virus,  as  the  staphylococci  found  in  Marinesco's  case  of  myehtis  occur- 
ring after  smallpox,  or  to  some  chemical  toxin,  the  product  of 
organisms),  and  the  evidence  of  an  influence  acting  throughout  the 
system,  afforded  by  the  wide  extent  of  the  disseminated  form  and  its 
occasional  association  with  disease  elsewhere,  such  as  optic  neuritis. 
It  is  to  this  form  that  our  knowledge  chiefly  relates.  Of  the  patho- 
logy of  simple  transverse  myelitis  we  have  only  the  fact  that  it 
often  follows  cold,  which  brings  it  into  closer  analogy  with  the  simple 
inflammations  of  other  organs. 

The  knowledge  that  has  been  gained  of  the  influence  of  toxic  blood- 
Btates  on  the  peripheral  nerves  gives  us  the  help  of  analogy  in  con- 
ceiving that  myehtis  may  often  have  the  same  origin.  But  a  difference 
exists  in  the  fact  that  the  multiple  neuritis  so  produced  is  commonly 
parenchymatous,  while  the  myehtis  is  conspicuously  interstitial.  Of 
the  pathology  of  parenchymatous  myelitis  we  know  almost  nothing. 
The  question  of  causation  will  be  again  alluded  to  in  considering  the 
special  inflammation  of  the  grey  matter. 

The  chief  relation  of  the  symptoms  to  the  lesion  has  been  con- 
sidered in  the  general  account  of  the  symptoms  of  spinal  cord  disease, 
and  those  relating  to  the  destructive  and  regenerating  processes  have 
been  mentioned  in  the  section  on  pathological  anatomy.  We  may, 
however,  further  note  that  the  vascular  changes,  which  take  so  large 
a  share  in  the  process  of  interstitial  inflammation,  as  seen,  for 
instance,  in  the  disseminated  form,  must  extend  the  process.  Tho 
escape  of  leucocytes  into  the  sheaths  tends  to  interfere  with  the 
liimen  of  the  vessel  and  the  flow  through  it,  while  their  accumula- 
tion in  the  tissue  must  entail  local  destruction  of  the  nerve-elements. 
If  the  cause  of  the  myelitis  is  a  persistent  influence,  such  as  the 
poison  of  a  progressing  specific  disease,  or  the  agent,  whatever  it 
be,  that  is  effective  in  gout,  it  is  easy  to  understand  that  the  local  in- 
fluence of  the  lesion  itself  will  co-operate  with  that  of  its  cause,  and 
induce  the  progressive  tendency  that  is  a  characteristic  of  this  foi'm. 

Diagnosis.  —  Myelitis  is  recognised  by  the  rapid  onset  of  symptoms 
indicating  structural  disease  of  the  cord.     Among  these  the  failure  of 


ACUTE    MYELITIS,  369 

power  is  tie  most  sieniflcant,  althongli  tLe  subjective  sensations  tliat 
accompany  it  are  oi^a  more  obtrusive  at  the  onset,  and  prevent 
mistake  as  to  the  meaning  of  the  weakness.  When  they  are  absent 
the  enfeeblement  may  be  mistaken  for  mere  prostration  in  cases  in 
which  some  general  illness  coincides,  and  it  is  probable  that  slight 
myelitis  sometimes  occurs  during  an  acute  specific  disease  and  alto- 
gether escapes  detection.  But  among  the  early  symptoms  no  one  is 
more  important,  for  its  definite  significance,  than  retention  of  urine ; 
and  its  importance  is  increased  by  the  fact  that  it  may  precede  all 
symptoms  in  the  legs  and  give  a  warning,  the  heed  of  which  might 
sometimes  enable  lasting  palsy  to  be  prevented  or  life  itself  preserved 
The  diagnosis  may  be  aided  by  the  presence  of  such  general  sym- 
ptoms as  attend  the  occurrence  of  inflammation  in  other  organs ;  but 
the  absence  of  these  is  of  little  negative  significance,  while  the 
presence  of  constitutional  disturbance  is  as  likely  to  mislead,  as  it  is 
to  suggest  a  local  affection.  The  position  of  the  myelitis  must  be 
inferred  from  the  considerations  already  described.  Its  upper  limit 
is  indicated  by  the  upper  limit  of  the  paralysis  ;  that  of  sensation  is 
most  readily  defined,  and  usually  corresponds  to  that  of  motion.  But 
the  upper  limit  no  more  shows  the  extent  of  the  disease  below  it,  than 
the  surface  of  water  does  its  depth.  The  extent  downwards  must 
be  gauged  by  the  impairment  of  the  functions  of  the  cord  as  a 
central  organ  (reflex  action  and  muscular  excitability)  in  the  parts 
paralysed,  while  the  degree  to  which  the  various  structures  of  the 
cord  are  damaged  must  be  inferred  from  the  character  and  degree 
of  the  symptoms  in  the  affected  parts.  In  ascertaining  the  state  of 
the  lower  dorsal  region  the  trunk  and  cremasteric  reflexes  are  espe- 
cially important. 

Thus  if  called  to  a  patient  who  has  rapidly  become  paraplegic,  the 
practitioner  shotdd  first  note  the  degree  of  motor  and  sensory  para- 
lysis of  the  legs  (indicating  whether  the  lesion  is  total  or  partial), 
and  how  high  up  the  trunk  the  symptoms  extend.  He  should  then 
test  the  reflex  action  in  the  affected  limbs  and  trunk,  and  ascertain 
the  state  of  myotatic  irritability  in  the  limbs.  These  indicate  the 
condition  of  the  reflex  arcs  in  the  lower  portion  of  the  cord.  Further 
information  on  this  point  is  afforded  by  the  state  of  muscular  nutri- 
tion, and  especially  by  the  evidence  of  the  state  of  nutrition  of  the 
nerve-fibres  which  is  revealed  by  faradism.  If  reflex  action  is  per- 
fect, and  the  muscles  have  preserved  their  tone,  this  examination  is  a 
matter  rather  of  scientific  interest  than  of  practical  importance.  It  is 
useless  to  apply  this  test  until  five  or  seven  days  after  the  onset, 
because  four  or  five  days,  and  often  eight  or  ten,  elapse  before  the 
degenerative  changes  occur  in  the  nerve-fibres.  The  examination  may 
then  be  made  without  any  risk,  provided  a  very  gentle  current  is 
employed,  just  sufficient  to  cause  a  contraction  in  the  corresponding 
muscles  of  a  healthy  limb,  and  the  current  need  not  be  appKed  to 
each  spot  for  more  than  a  second.  The  isolated  faradic  shock  may 
VOL.  I.  24 


370  SPINAL   CORD. 

be  employed  with  advantage  on  account  of  tlie  absence  of  the  strong 
sensory  stimulation  that  is  caused  by  the  current  (see  "  Atrophic  Para- 
lysis ") .  If  no  change  is  found,  the  examination  may  be  repeated  at 
the  end  of  ten  days  from  the  onset.  If  no  muscles,  at  the  end  of  that 
time,  present  any  considerable  diminution  of  contractility,  it  shows 
that  no  considerable  nutritive  change  is  taking  place  in  the  nerves, 
and  that  the  grey  matter  from  which  the  nerves  proceed  is  not 
inflamed.  On  the  other  hand,  if  certain  muscles  present  a  diminu- 
tion of  irritability,  others  being  normal,  there  is  a  focal  lesion  in  the 
corresponding  grey  matter.  If  all  the  muscles  of  both  legs  present 
such  a  failure,  there  is  inflammation  of  the  lumbar  grey  matter, 
and  if  sensation  is  also  lost,  a  total  lumbar  myehtis.  So,  if  there  is 
cervical  myelitis  with  paralysis  of  all  four  limbs,  the  condition  of 
irritability  of  the  arm  muscles  shows  whether  the  disease  involves,  or 
is  above,  the  grey  matter  in  the  lower  half  of  the  cervical  enlarge- 
ment. If  there  is  impairment  of  irritability,  its  extent  in  the  two 
arms  will  afford  an  indication  as  to  whether  the  myelitis  is  total,  or 
whether  it  affects  chiefly  certain  spots.  The  distinction  from  simple 
polio-myelitis  depends  on  the  fact  that  the  symptoms  are  not  purely 
motor.  It  is  only  when  the  myelitis  is  cervical  or  lumbar  that  the 
diagnosis  is  a  matter  of  difficulty ;  in  these  cases  the  arms  and  legs 
are  the  seat  of  atrophic  paralysis  in  ordinary  myelitis,  because  the 
grey  matter  is  involved.  There  is,  indeed,  polio-myelitis  in  such  cases, 
but  there  is  also  more,  and  the  impairment  of  sensation,  together  with 
the  simple  palsy  of  the  legs  when  the  disease  is  in  the  cervical  region, 
shows  the  involvement  of  other  parts  than  the  anterior  cornua, — which 
is  the  essential  distinction.  The  most  equivocal  condition  is  that 
which  exists  when  simple  but  severe  polio -myelitis  in  the  cervical 
region  spreads  to  the  white  columns  immediately  contiguous,  and 
causes  weakaess  of  the  legs.  But  it  never  causes  complete  loss  of 
power,  even  for  a  short  time,  and  the  legs  soon  recover,  whereas  in 
ordinary  cervical  myelitis  the  loss  of  power  in  the  legs  is  often  com- 
plete and  prolonged,  and  is  accompanied  by  impairment  of  sensation, 
not  met  with  in  polio-myelitis. 

The  distinction  of  the  other  varieties  of  myelitis  rests  on  the 
special  features  ab-eady  described.  That  of  the  disseminated  form 
rests  on  the  irregular  distribution  of  the  symptoms  and  the  evidence 
of  damage  extending  through  a  considerable  vertical  extent  of  the 
cord,  but  in  some  parts  incomplete  in  degree.  An  inflammation 
which  contiaues  to  extend  after  the  first  two  or  three  days  is  certainly 
disseminated,  and  most  subacute  cases  are  of  this  variety,  and  so  are 
those  that  are  secondary  to  blood-states.  The  distraction  is  impor- 
tant, because  this  form  is  far  more  grave  than  any  other,  and  more 
likely  to  cause  death.  It  is  doubtful  whether  central  myelitis  can  be 
diagnosed  during  life  ;  there  is  usually  a  slighter  degree  of  myelitis 
in  the  other  elements  which  causes  symptoms  resembling  the  ordinary 
form.     There  is,  howevei,  one  exception  to  the  rule  that  a  spreading 


ACUTE    MYELITIS.  JJ71 

myelitis  is  disseminated ;  tlie  diffuse  transverse  form  that  is  secon- 
dary to  some  other  morbid  processes,  either  to  compression  from 
without  or  to  haemorrhage  within  the  cord,  often  undergoes  sub- 
sequent extension.  Acute  myelitis  in  syphilitic  subjects,  even  if  due 
to  syphilis,  presents  no  distinguishing  features,  nor  is  its  coxurse  in- 
fluenced by  antisyphilitic  treatment.  But  the  latter  fact  is  true  of 
all  acute  syphilitic  inflammations — the  tissue  destroyed  by  the  process 
cannot  be  restored  by  the  removal  of  the  cause.  In  rare  cases,  how- 
ever, an  acute  parenchymatous  myelitis  in  a  syphilitic  subject  is 
suggested  by  the  limited  impairment  of  a  certain  f miction.  In  such 
cases,  and  in  others  in  which  the  symptoms  point  to  a  toxic  blood- 
state  acting  on  the  nerve-elements  without  destruction  of  tissue,  the 
effect  of  treatment  seems  to  confirm  the  diagnosis. 

If  transverse  myelitis  has  been  diagnosed,  the  question  should 
always  be  asked.  Is  it  primary  or  secondary  to  some  other  process, 
of  which  the  most  common  is  compression  of  the  cord  ?  We  have 
seen  that  myelitis  may  result,  even  in  acute  form,  from  external 
pressttre,  which  may  be  that  of  a  growth  or  of  disease  of  the  bone. 
The  indications  are,  first,  the  fact  that  root-pains  at  the  level  of  the 
disease  preceded  the  myelitis  for  at  least  some  weeks  ;  and  secondly, 
the  direct  evidence  of  a  disease,  as  caries,  or  cancer  of  the  spine, 
to  which  the  inflammation  may  be  secondary.  The  spinal  column 
should  be  carefully  examined  in  all  cases,  not  merely  once,  but  again 
and  again.  When  the  myelitis  is  developed  there  may  be  no  signs  of 
bone  disease,  and  yet  these  may  appear  in  the  course  of  a  few  weeks 
or  months.  Myelitis  in  a  cancerous  patient  should  always  suggest  a 
secondary  growth  in  the  spine.  A  woman,  shortly  after  the  removal 
of  a  cancer  from  the  breast,  became  paraplegic ;  no  evidence  of  bone 
disease  could  at  first  be  found,  but  in  a  few  weeks  the  vertebral 
column  became  distinctly  enlarged  from  secondary  cancer.  This 
element  in  diagnosis  has  become  of  great  importance  from  the 
certainty  that  some  simple  external  growths  can  be  removed. 

The  diagnosis  from  other  lesions  of  the  cord  is  chiefly  by  the  mode 
of  onset.  In  hasmorrhage  the  symptoms  develop  in  a  few  minutes,  and 
it  is  chiefly  in  the  cases  of  hsemorrhagic  myelitis  that  any  diagnostic 
difficulty  presents  itself.  The  sudden  paralysis  is  then  commonly 
preceded  by  slight  sensory  symptoms,  tingling,  &c.,  and  sometimes  by 
fever.  If  these  are  absent  in  cases  of  actually  sudden  onset,  primary 
myelitis  is  far  less  likely  than  haemorrhage.  The  acute  spinal  pain 
that  is  common  in  haemorrhage  is  absent  in  myelitis.  If  an  onset 
indicating  haemorrhage  is  followed  by  a  gradual  extension  of  the  sym- 
ptoms during  the  next  twelve  or  twenty-four  hours,  myelitis  secondary 
to  haemorrhage  may  be  inferred. 

When  acute  myelitis  ascends  the  cord,  so  that  the  legs,  muscles  of 
the  trunk,  and  the  arms  are  successively  paralysed,  its  course  resem- 
bles that  of  acute  ascending  paralysis,  or  "  Landry's  paralysis,"  in 
which  no  lesion  of  the  cord  is  found  after  death.    The  most  important 


372  SPINAL    COED. 

distinction  is  tiiat  ra  ascending  myelitis  sensation  is  affected,  and 
if  the  patient  survives  there  is  a  strong  tendency  to  trophic  disturb- 
ance in  the  skin,  while  many  muscles  waste  and  present  loss  of  f  aradic 
irritability.  In  acute  ascending  paralysis,  on  the  other  hand,  sensa- 
tion is  little  impaired ;  some  parts  may  be  spared  by  the  ascendiag 
palsy ;  bedsores  do  not  form ;  and,  if  the  case  is  not  fatal,  there  is  no 
change  in  the  electric  irritability  of  the  muscles. 

In  meningitis  the  symptoms  of  irritation,  severe  pains,  muscular 
rigidity,  &c ,  are  prominent,  while  they  are  absent  in  simple  myelitis 
But  in  many  cases  the  two  conditions  co-exist,  and  which  disease  is 
predominant  can  only  be  decided  by  the  order  and  degree  of  the 
development  of  the  symptoms.  In  meningeal  hsemorrhuge  there  is 
severe  pain  in  the  back  and  acute  irritation  of  the  nerve-roots. 

The  distinction  from  multiple  neuritis  has  been  considered  in  the 
account  of  that  disease.  It  chiefly  arises  in  the  cases  of  polio-myelitis, 
presently  to  be  considered ;  but  a  difficulty  may  exist  when  a  paren- 
chymatous myelitis  affects  the  structures  on  which  co-ordinatiou 
depends,  and  produces  the  condition  of  myelitic  ataxy.  Such  cases 
are  distiaguished  from  those  of  neuritic  pseudo-tabes  by  the  fact 
that  some  other  structures  are  involved,  so  that  symptoms  are 
present  that  are  only  met  with  in  diseases  of  the  spinal  cord.  A  very 
acute  onset,  so  that  the  symptoms  reach  a  high  degree  in  a  few  hours, 
is  also  evidence  of  the  myelitic  origia.  The  case  mentioned  on.  p.  366 
affords  an  illustration  of  these  distinctions. 

Myelitis  is  far  from  rare  in  patients  of  the  age  and  sex  ia  which 
hysteria  especially  prevails,  and  many  cases  are  mistaken  for  hysterical 
paraplegia — sometimes  because  symptoms  of  hysteria  concur,  often 
merely  because  the  patient  is  a  girl  and  her  legs  well  nourished.  The 
converse  error  is  very  rare.  The  mistake  occurs  especially  in  cases  of 
transverse  dorsal  myelitis,  ia  which  there  is  no  wasting  of  the  muscles 
of  the  legs.  The  gradual  development  of  considerable  excess  of 
myotatic  irritability  is  a  symptom  of  great  diagnostic  value,  and 
when  this  increases  to  characteristic  extensor  spasm  there  should  be 
no  room  for  doubt.  If  one  leg  is  hfted  from  the  bed,  and  the  other 
moves'  with  it  owing  to  the  rigid  extensor  spasm,  organic  disease  ia 
certain ;  hysterical  contracture  never  fixes  the  legs  to  the  pelvis  so  as 
to  permit  this  effect.  In  hysterical  paraplegia  there  may  be  retention 
of  urine,  but  there  is  not  incontinence.  Among  other  conclusive 
symptoms  of  organic  disease,  a  girdle  pain,  and  incontinence  of  faeces, 
are  of  especial  value.  Trophic  changes  in  the  sMn  sometimes  decide, 
even  alone,  the  nature  of  the  case. 

PROGNOSIS. — The  primary  danger  to  life  in  myelitis  depends  upon 
the  risk  of  respiratory  palsy,  and  hence  on  the  region  of  the  cord  ia 
which  the  disease  is  situated.  A  disposition  to  spread  is  always  of 
grave  significance,  and  so  also  are  indications  of  a  serious  blood- 
change,  which  is  likely  to  increase  the  damage  it  has  caused.  On 
this  account  the  prognosis  is  worse  in  disseminated  than  in  simple 


ACUTE    MYELITIS.  873 

transverse  myelitis,  and  it  is  longer  before  confidence  can  be  felt  that 
arrest  will  endure.  The  risk  is  great  whenever  the  cervical  region  ia 
diseased,  so  that  the  intercostal  muscles  are  paralysed,  and  in  propor- 
tion to  the  nearness  of  the  lesion  to  the  origin  of  the  phrenic  nerve, 
on  which  life  then  depends.  Hence  a  careful  examination  of  the 
respiratory  movements  should  be  the  first  concern.  A  ny  indications 
that  foci  of  inflammation  have  developed  in  the  medulla  oblongata 
are  of  extremely  grave  significance.  Another  danger  is  from  acute 
trophic  disturbances,  and  on  this  account  the  prognosis  must  be 
guarded  whenever  the  lumbar  enlargement  is  diseased,  or  is  tiireatened 
by  the  extension  of  the  inflammation.  Hence  bedsores,  if  forming 
early,  within  the  first  month,  are  an  unfavorable  indication ;  at  a  later 
period  they  have  less  influence  on  the  prognosis.  There  may  even  be 
a  phlegmonous  condition  of  the  viscera  produced.  The  occurrence  of 
cystitis,  and  especially  of  any  indication  of  secondary  kidney  disease, 
increases  very  much  the  gravity  of  the  case. 

The  prospect  of  recovery  of  power  chiefly  arises  when  the  onset  ia 
•over,  and  the  disease  has  become  stationary.  Its  degree  depends  (1) 
on  the  intensity  of  the  disease,  as  shown  especially  by  the  loss  of  sen- 
sation as  well  as  of  motion,  and  (2)  on  its  vertical  extent  as  indicated 
by  the  impairment  of  the  central  functions  of  the  cord ;  (3)  on  the  early 
•occurrence  of  symptoms  of  improvement.  The  longer  loss  of  sensa- 
tion continues,  the  less  is  likely  to  be  the  degree  of  ultimate  recovery. 
(4)  It  is  influenced  to  some  extent  by  the  cause  of  the  myelitis,  being 
better  if  this  is  due  to  a  removable  cause,  such  as  pressure,  than  if 
spontaneous.  (5)  It  is  better  when  the  disease  is  confined  to  the 
dorsal  region  than  when  rapid  atrophy,  &c.,  of  the  muscles  show  that 
the  lumbar  grey  matter  is  involved.  The  longer  motor  palsy  remains 
absolute,  the  less  perfect  >,l11  be  the  ultimate  recovery.  If  some 
power  returns  within  a  fortnight,  the  amount  that  will  be  regained 
■will  probably  be  great ;  but  even  complete  loss  of  power  for  six  months 
does  not  preclude  the  ultimate  return  of  the  ability  to  walk,  and  I 
have  even  known  this  result  after  complete  paralysis  for  a  year  from 
transverse  myelitis.  The  development  of  increased  myotatic  irrita- 
bility shows  that  improvement  wiU  not  speedily  occur,  but  even 
rigidity  and  the  state  of  "  spastic  paraplegia  "  does  not  lessen  materi- 
ally the  prospect  of  some  recovery  in  a  case  in  which  the  loss  of  power 
remains  complete  for  a  month  or  more.  Indeed,  the  spasm  often 
enables  the  patient  to  stand  with  a  slighter  degree  of  volimtary  power 
than  would  suffice  if  the  limbs  were  supple. 

The  fact  of  preceding  syphilis  must  be  allowed  very  little  influence 
on  the  prognosis  ia  a  case  of  simple  acute  myelitis,  but  greatly  im- 
proves the  prognosis  i£  there  are  indications  that  the  inflammation  is 
secondary  to  a  more  chronic  process  outside  the  cord. 

Treatment. — If  a  case  comes  tinder  observation  at  the  earliest 
period,  when  only  slight  sensory  disturbance  and  slight  weakness  of 
the  legs  indicate  the  commencing  process,  the  question  arises  whether 


874  SPINAL    COED. 

any  treatment  can  avert  the  further  development  of  the  inflammation. 
If  the  symptoms  are  clearly  due  to  exposure  to  cold,  a  hot  bath, 
followed  by  free  diaphoresis,  should  be  employed,  and  followed  by 
counter-irritation  and  the  other  measures  now  described.  If  con- 
siderable paralysis  shows  that  the  process  of  inflammation  is  fulJy 
developed,  little  can  be  expected  from  these  measures,  and  it  is 
better  not  to  subject  the  patient  to  treatment  that  is  incompatible 
with  perfect  rest.  This,  in  all  cases,  is  of  paramount  importance. 
Both  functional  excitation  of  the  cord  and  movement  of  the  spinal 
column  should  be  avoided.  The  remarks  regarding  posture  made  in 
the  account  of  the  treatment  of  inflammation  of  the  membranes 
apply  also  o  that  of  the  cord  itself  ;  it  is  most  undesirable  that  the 
spine  should  be  the  lowest  part  of  the  body,  and  it  is  rather  less 
diffictdt  to  keep  the  patient  off  the  back  in  myelitis  than  in  menin- 
gitis. A.  plank  back-rest  in  the  bed  will  be  found  a  great  assistance 
in  securing  comfortable  rest  on  the  side.  If  there  is  any  reason  to 
suspect  haemorrhage,  or  if  there  are  indications  of  rapid  extension  of 
the  inflammation,  the  prone  position  should  be  adopted,  and  even  in 
myelitis  it  is  well  to  adopt  it  at  times  as  a  change  from  the  lateral 
posture. 

The  removal  of  blood  from  the  skin  of  the  back  over  the  affected 
region,  by  leeches  or  wet  cupping,  is  an  old  measure,  which  finds  some 
theoretical  justification  in  the  fact  that  the  blood  from  the  structures 
behind  the  spine  passes  into  the  same  veins  as  the  blood  from  the 
spinal  cord  itself.  Hence  this  measure  may  conceivably  have  some 
influence  on  the  circulation  in  the  cord.  If  the  patient's  strength  is 
not  such  as  to  render  the  abstraction  of  blood  desirable,  dry  cupping 
may  be  employed,  or  the  vessels  of  the  skin  may  be  dilated  by  hot 
fomentations,  or  a  mustard  plaster,  or  hot  water  bags.  By  stimu- 
lating the  <  utaneous  nerves,  these  agents  may  also  influence,  in  a 
reflex  manner,  the  vessels  of  the  cord.  The  application  of  cold  to  the 
spine,  as  by  a  spinal  ice-bag,  has  also  been  recommended.  Conti-ciry  as 
these  therapeutic  agents  seem,  it  is  probable  that  each  moderates  local 
inflammation  in  the  same  manner,  by  causing  first  contraction  and  then 
dilatation  of  the  vessels  of  the  inflamed  part,  and  so  lessening  the 
tendency  to  stasis  of  the  blood,  on  which  some  effects  of  inflammation 
depend.  Unless  there  is  reason  to  suspect  haemorrhage,  the  applica- 
tion of  warmth  is  the  safer  and  probably,  judging  from  experience, 
the  more  effectual.  At  the  very  onset  of  inflammation  mild  counter- 
irritation  is  unquestionably  useful,  and  even  a  blister  may  be  em- 
ployed. The  actual  cautery  of  Paquelin,  applied  in  a  series  of  spots 
along  each  side  of  the  spine,  is  probably  more  effective,  and  less  liable 
to  set  up  or  intensify  trophic  disturbance ;  but  when  the  process  has 
reached  a  considerable  degree  it  is  very  doubtful  whether  counter- 
irritation  has  much  influence  until  the  acute  stage  is  over. 

In  other  respects  the  treatment  of  acute  inflammation  of  the  cord 
must  be  guided   rather  by  the  nature  of   the  process   than   by  the 


ACUTE    MYELITIS.  875 

chaiacter  of  the  organ  in  whicTi  it  occurs,  and  the  fact  that  it  is  an 
acute  local  inflammation  should  be  kept  in  view.  A  nutritious  hut 
unstimulating  diet,  aperients,  and  diuretics  are  desirable  in  all  cases. 
If  there  is  constipation  a  free  purgative  may  be  given.  Whenever 
there  is  evidence  of  a  morbid  blood-state,  it  is  important  that  this 
should,  if  possible,  be  improved,  but  we  have  still  to  learn  how  most 
toxsemic  states  can  be  neutralised.  ISTitrous  ether  may  be  given  as  a 
diuretic  with  some  tincture  of  digitalis,  which  tends  to  render  the 
circulation  uniform,  and  to  lessen  stasis  by  its  influence  on  the  small 
arteries.  The  reason  for  diuresis  is  that  probably  no  local  inflamma- 
tion occurs  that  is  not  associated  with  a  morbid  state  of  the  blood, 
which  may  be  to  some  extent  relieved  by  the  action  of  the  kidneys. 
If  any  special  drug  is  given,  it  may  be  combined  with  those  above 
named.  It  is  as  difficult  to  ascertain  the  effect  of  the  drugs  which 
are  supposed  to  exert  a  special  influence  on  myelitis,  as  it  is  in  the 
case  of  other  local  inflammations,  which  have  no  predetermined 
degree,  and  tend  to  subside  when  they  have  reached  their  height. 
Ergot  was  recommended  by  Brown- Sequard,  chiefly  on  theoretical, 
grounds,  and  has  been  extensively  employed.  In  rare  cases  it  has 
seemed  to  do  good.  In  cases  of  haemorrhagic  myelitis  it  may  reason- 
ably be  given  with  greater  confidence,  or  ergotin  (3  to  5  grs.)  may  be 
injected  beneath  the  skin.  Belladonna  has  also  been  recommended, 
but  the  evidence  that  it  influences  the  morbid  process  is  not  strong. 
Mercury  has  been  largely  employed,  given  by  the  mouth  and  by 
inunction.  The  influence  of  mercury  on  the  inflammation  of  internal 
organs  does  not  seem  so  great  as  upon  that  of  the  fibrous  tissues  and 
of  the  structures  that  invest  organs.  Certainly  in  myelitis  its  effect 
is  less  distinct  than  it  is  in  many  cases  of  meningitis.  Iodide  of 
potassium  seems  to  be  no  more  efficacious  than  mercury.  In  eases  of 
transverse  myelitis  occurring  in  syphilitic  subjects  the  treatment  for 
syphilis  seems  to  have  but  little  influence  on  the  morbid  process.  It 
is  true  that  such  treatment  is  rarely  adopted  at  the  very  onset  of 
acute  myelitis,  but  after  the  disease  has  developed  energetic  treatment 
does  not  seem  to  modify  its  subsequent  course.  This  is  true  also  of 
the  subacute  disseminated  myelitis  that  occurs  in  the  subjects  of 
syphiHs,  and  might  be  expected  to  be  more  amenable.  I  have  known 
this  form,  concurring  with  syphilitic  disease  of  the  cerebral  arteries, 
to  develop  and  run  its  course  to  a  fatal  termination  in  spite  of  con- 
tinuous antisyphiHtic  treatment,  to  which  the  arteritis  yielded. 

In  the  general  management  of  a  case  of  myelitis,  two  points  are  of 
extreme  importance.  One  is  to  avoid,  by  scrupulous  cleanliness  and 
care,  the  exciting  causes  of  bedsores.  The  skin  should  be  most  care- 
fully watched,  and  any  indication  of  deleterious  pressure  met  by  a 
change  of  position  or  an  alteration  of  the  mode  of  supporting  the  part. 
Cotton  wool  is  very  useful  for  this  purpose.  If  there  is  a  marked 
tendency  to  trophic  changes  the  j)atient  should  be  placed  on  a  water- 
bed.     When  there  is  incontiaence  of  urine,  the  difficulty  of  avoiding 


376  SPINAL    CORD. 

irritation  of  tlie  skin  is  greatly  increased.  For  males,  a  bed  urinal  is 
sometimes  useful,  but  often  it  causes  irritation  and  even  sloughing  of 
tbe  prepuce,  and  tben  does  more  harm  than  good,  A  quantity  of 
boracic  or  salicylic  absorbent  cotton  wool,  changed  as  often  as  it 
becomes  saturated,  is  one  of  the  best  means  of  meeting  this  difficulty. 
It  must  be  remembered  that  the  prevention  of  bedsores  is  the  pre- 
vention of  one  common  cause  of  death.  Should  offensive  sores  form, 
a  quantity  of  picked  oakum,  placed  outside  the  immediate  dressing,  is 
a  cheap  and  most  effective  means  of  preserving  the  air  of  the  room 
from  the  foetor  of  the  sores,  and  is  also  a  useful  substitute  for  absor- 
bent cotton  wool,  in  the  case  of  the  poor,  to  absorb  the  urine  or  receive 
faeces  that  are  passed  unconsciously.  Such  material  is  also  useful  for 
relieving  pressure  when  a  water-bed  cannot  be  obtained.  The  second 
point  in  management  is  the  treatment  of  retention  of  urine.  If  there 
is  either  simple  retention  or  overflow  incontinence  the  bladder  must 
be  regularly  emptied  by  the  catheter,  great  care  being  exercised  to 
prevent  the  introduction  of  contaminating  germs.  The  importance  of 
daily  examining  the  abdomen  to  see  that  retention  has  not  occurred, 
cannot  be  exaggerated.  If  the  bladder  is  left  full  and  the  urine 
allowed  to  dribble  away,  inflammation  is  sure  to  be  set  up,  and 
probably  also  pyelo-nephritis.  If  cystitis  occurs,  antiseptic  washes 
must  be  used  to  lessen,  as  far  as  possible,  the  decomposition  of  the 
urine.  Under  this  influence  the  cystitis  usually  lessens,  and  one  grave 
danger  to  life  is  obviated. 

When  the  disease  of  the  cord  has  become  stationary,  the  patient 
may  be  allowed  to  move,  and  a  more  tonic  treatment  may  be  adopted. 
Iron,  quinine,  or  arsenic  may  be  given.  Strychnia  must  be  given  only 
in  very  small  doses  if  there  is  any  excess  of  reflex  action.  Occasional 
counter-irritatiop  may  be  employed,  repeated  frequently  if  any  im- 
provement seems  to  result.  The  limbs  may  be  rubbed,  and  any 
muscular  wasting  treated  with  electricity.  It  is  not  desirable  to  use 
electricity  as  a  therapeutic  agent  while  the  cord  disease  is  in  an  acutely 
active  stage.  There  is  no  evidence  that  the  application  of  electricity 
to  the  spinal  column  has  any  influence  on  the  process  of  recovery  of 
the  cord.  Its  chief  value  is  to  maintain,  as  far  as  possible,  the 
nutrition  of  any  muscles  of  which  the  nerves  have  undergone  de- 
ge'neration.  In  cases  of  dorsal  myelitis,  in  which  the  legs  are  well 
nourished,  and  the  reflex  action  is  excessive,  it  is  better  not  to 
apply  any  form  of  electricity.  The  unavoidable  stimulation  of  the 
sensory  nerves  tends  to  increase  the  reflex  over-action.  Careful  atten- 
tion should,  in  all  cases,  be  paid  to  the  position  of  the  limbs  during 
the  stage  of  helplessness,  so  as  to  avoid  as  far  as  possible  the  develop- 
ment of  contractions.  Tor  the  condition  of  active  spasm,  which  often 
develops  after  severe  myelitis,  not  much  can  be  done;  such  special 
treatment  as  can  be  adopted  is  described  in  the  chapters  on  Primary 
Spastic  Paraplegia. 


ABSCESS   OF   THE   COED.  877 


Abscess    of   the   Spinal   Coed. 

Simple  inflammation  of  tlie  spinal  cord  scarcely  ever  goes  on  to 
the  formation  of  pus,  although  leucocytes  may  accum-"  .e  at  certain 
points  of  the  grey  matter  so  densely  as  to  constitute  microscopic 
collections  of  pus,  and  in  very  rare  cases  of  this  kind  such  minute 
abscesses  have  been  sufficiently  large  to  be  visible  to  the  naked  eye. 
Pus  only  forms  in  the  substance  of  the  cord  in  considerable  quantity 
in  cases  of  purulent  meningitis.  In  most  instances  the  purulent 
meningitis  has  been  of  septic  origin,  rarely  traumatic.  In  the  former 
case  suppuration  within  the  brain  may  coincide  with  that  in  the 
Bpinal  cord,  and  in  the  latter  it  may  be  very  extensive,  and  occur  at 
more  than  one  spot.  The  symptoms  are  those  of  an  acute  irritative 
myehtis,  but  they  are  often  lost  in  those  of  the  purulent  meningitis 
which  precedes  the  disease  of  the  cord  itself.  Their  special  feature  is 
their  association  with  a  cause  of  septic  suppuration,  as  well  as  with  a 
high  temperature  and  other  symptoms  of  a  septic  blood-state. 

A  good  example  of  the  disease  is  a  case  recorded  by  Nothnagel. 
A  patient  suffering  from  cough  and  most  offensive  expectoration  was 
suddenly  seized  with  severe  pains  on  both  sides  of  the  abdomen, 
attended  by  a  sense  of  constriction  and  quickly  followed  by  paralysis 
of  the  bladder  and  of  the  legs,  with  loss  of  sensation  and  of  reflex 
action.  An  abscess  of  the  cord  was  diagnosed.  After  death  there 
was  extensive  purulent  spinal  meningitis,  and  the  dorsal  and  lumbar 
cord  contained  an  extensive  collection  of  gangrenous  offensive  pus, 
which  seemed  to  occupy  the  central  part  of  the  cord,  from  the  cervical 
enlargement  downwards.  Some  abscesses  were  found  also  in  the 
white  substance  of  the  brain.  In  another  case  described  by  Ullman* 
two  extensive  foci  of  suppuration  existed  in  the  cord,  one  cervical,  the 
other  lumbar ;  the  former  had  caused  extensive  destruction  of  tissue, 
and  the  pus  had  escaped  into  the  subdural  space.  The  affection  was 
supposed  to  be  secondary  to  gonorrhoea. 


Embolism   op   the   Spinal   Coed. 

Tlie  occurrence  of  embolism  in  the  spinal  cord  has  not  yet  been 
proved,  but  a  few  cas^s  have  been  met  with  which  suggest  the 
possibility  that  the  process  has  been  the  exciting  cause  of  an  acute 
myelitis.  In  a  young  man  with  mitral  regiirgitation,  considerable 
weakness  of  the  right  leg  came  on  suddenly — in  a  moment — with 
transient  spasm.  The  onset  indicated  a  sudden  lesion  in  ■  the  cord, 
which  might  well  have  been  the  embolic  obstruction  of  a  small  vessel. 
In  a  case  recorded  by  Weiss,  a  boy  aged  sixteen,  with  chronic  mitral 
disease,  was  suddenly  seized  with  complete  paraplegia,  followed  by 

*  '  Zeitschr.  f .  kl.  Med.,'  xvi,  1889. 


378  SPINAL    CORD. 

bedsores,  &c.  He  died  four  montlis  after  the  onset,  and  the  lumbar 
enlargement  was  found  completely  softened,  with,  old  coagula  in  the 
arteries.  There  was  embolism  of  the  kidneys  and  spleen,  and  the 
cortex  of  each  cerebral  hemisphere  presented  small  foci  of  softening. 
Such  cases  justify  a  suspicion  that  embolism  may  be  the  cause  of  a 
sudden  lesion  of  the  cord  in  a  patient  in  whom  a  source  of  embolism 
exists,  and  the  process  has  occurred  in  other  organs. 


Cheonic    Myelitis. 

The  spinal  cord  may  be  the  seat  of  chronic  inflammation,  wliicli 
develops  slowly,  as  such,  in  the  course  of  a  few  or  many  months  ;  and 
the  condition  may  also  occur  as  the  sequel  to  acute  myelitis,  which, 
instead  of  subsiding,  may  persist,  manifesting  from  time  to  time  signs 
of  activity.  It  is  often  difficult  to  sa^y  whether  such  a  condition 
should  be  regarded  as  an  acute  myelitis  that  has  not  subsided,  or  a 
chronic  inflammation  beginning  acutely.  In  many  instances,  indeed, 
it  is  probable  that  the  disease  is  most  accurately  regarded  as  a 
combined  form  in  which  the  causes  of  both  are  operative,  and  a 
subsiding  acute  inflammation  is  arrested  and  maintained  by  the 
influences  that  would  be  capable  of  inducing  a  primary  chronic 
myelitis.  Such  progressive  tendency  is  especially  conspicuous  in  the 
disseminated  form,  in  which  the  foci  of  inflammation  may  remain,  and 
fresh  ones  may  develop  from  time  to  time  in.  a  chronic  or  subacute 
manner. 

The  lesions  of  chronic  myelitis  resemble  those  of  acute  myelitis  in 
seat  and  distribution,  and  intermediate  cases  connect  them  by  analogous 
subacute  forms.  They  differ  from  acute  inflammation  both  in  the 
longer  time  occupied  in  their  development,  and  in  the  absence  of  the 
considerable  vascular  disturbance  which  forms  part  of  the  acute 
process.  Such  chronic  myelitis  may  be  focal,  disseminated,  or  diffuse. 
In  the  former  case  it  may  involve  the  whole  thickness  of  the  cord  at  a 
certain  level — chronic  transverse  myelitis ;  or  only  part  of  it,  sometimes 
one  half,  occasionally  for  a  considerable  vertical  extent.  The  chronic 
disseminated  myelitis  may  resemble  the  corresponding  subacute  form 
in  distribution,  many  points  of  inflammation  being  scattered  through 
a  small  region,  or  through  a  large  part  of  the  cord,  and  its  symptoms 
may  become  diffuse  by  the  union  of  the  effects  of  the  disease. 

The  term  "  chronic  myelitis  "  has,  however,  been  used  in  a  much 
wider  sense,  and  has  even  been  applied  by  some  to  all  local  processes 
attended  by  an  increase  of  interstitial  tissue,  whether  this  is  primary 
or  is  merely  secondary  to  a  degeneration  of  the  nerve-elements.  Thus 
the  degeneration  of  the  nerve-cells  in  progressive  muscular  atrophy 
has  been  regarded  and  described  by  many  as  a  chronic  myelitis  of  the 
grey  substance,  notwithstanding  the  fact  that  there  is  usually  a 
degeneration  of  the  whole  motor  path,  in  both  segments,  from  the 


CHRONIC    MYELITIS.  379 

cortex  of  tlie  brain,  ttrotigh.  the  pyramidal  tracts  and  motor  nerves, 
and  that  the  anterior  cornua  merely  constitute,  as  it  were,  a  focus  in 
which  the  effects  of  the  processes  are  especially  conspicuous.  Such 
a  morbid  process  is  evidently  quite  different  in  nature  from  a  focus  of 
ordinary  inflammation,  in  which  the  primary  change  is  interstitial 
and  random  in  range  and  effects,  and  presents  no  limitation  to  func- 
tion in  the  incidence  of  the  consequences  on  the  nerve-structures. 
Whenever  this  latter  feature  can  be  traced,  the  malady  must  be 
regarded  as  a  "  system  disease,"  i.  e.  as  a  disease  affecting  a  system  of 
nerve- structures  that  have  a  common  function.  If  "  system  diseases  " 
are  forms  of  inflammation,  they  are  "  parenchymatous  inflammations ; " 
and  those  that  are  chronic  in  course  from  beginning  to  end  are  more 
commonly  termed  "  degenerations,"  and  are  so  classed  and  described 
in  these  pages.  Only,  then,  the  chronic  interstitial  processes  of  random 
position  and  influence  are  here  considered. 

Etiology. — The  causal  relations  of  chronic  myelitis,  even  as  thus 
limited  in  conception,  are  very  various.  An  inherited  neuropathic 
tendency  can  sometimes  be  traced,  but  far  more  rarely  than  if  the 
cases  are  included  in  which  the  nerve-elements  suffer  primarily.  The 
disease  is  most  common  in  early  and  middle  adult  life,  but  it  occurs 
occasionally  in  old  age  ;  it  is  met  with  in  both  sexes,  and  is  perhaps 
more  prevalent  in  females  in  the  first  half  of  middle  life  and  in  males 
in  the  second — an  important  fact,  because  so  many  of  the  cases  in 
young  adult  women  are  thought  to  be  hysterical  in  nature. 

Various  conditions  which  lessen  constitutional  strength  predispose 
to  the  disease.  Definite  causes  often  cannot  be  traced,  although 
many  of  the  exciting  causes  of  acute  myelitis  seem  capable  of  giving 
rise  to  the  chronic  form,  when  acting  for  a  considerable  time,  or 
frequently  repeated,  or  in  specially  predisposed  persons.  Thus  while  a 
single  intense  exposure  to  cold  may  produce  acute  myelitis,  frequent 
habitual  exposure  may  cause  the  chronic  form.  Injury  is  a  frequent 
cause,  and  seems  to  act  in  more  than  one  way.  Chronic  inflammation 
may  develop  in  the  vicinity  of  a  damaged  spot,  when  the  traumatic 
lesion  may  have  been  too  slight  to  cause  pronounced  symptoms,  or 
may  have  caused  slight  effects  which  are  subsequently  lost  in  the 
more  severe  and  extensive  consequences  of  the  myelitis.  In  other 
cases  the  effects  of  the  lesion  may  be  too  slight  in  degree  to  be  at 
first  perceptible,  but  (either  by  a  slow  influence  on  the  processes  of 
nutrition,  or  by  extensive  minute  interstitial  lesions  that  induce 
secondary  traumatic  inflammation)  the  symptoms  of  extensive  damage 
to  the  cord  slowly  follow  an  injury  that  has  no  immediate  effect  on 
function.  These  cases  will  be  further  considered  in  a  subsequent 
chapter.  Repeated  over-exertion  has  also  seemed  effective  in  some 
cases.  Sexual  excess  has  been  thought  to  be  a  cause,  but  on  less 
clear  evidence. 

Chronic  myeUtis  may  be  secondary  to  other  local  morbid  processes, 
especially  to  adjacent  inflammation,  which,  when  it  also  causes  coxa- 


380  SPINAL   COED. 

pression,  invariaWy  gives  rise  to  clironic  myelitis ;  the  inflammation 
that  results  from  pressure  on  the  cord  may  be  either  chronic  or  acute. 
Another  series  of  causes  consists  of  constitutional  states  capable  of 
giving  rise  to  local  inflammations.  Chronic  alcoholism  is,  perhaps, 
the  most  influential  and  important  of  these.  Chronic  myelitis  due 
to  it  is  usually  associated  with  chronic  meningitis,  and  most  intense 
at  the  periphery,  beneath  the  inflamed  membranes;  but  it  thence 
extends  into  the  substance  of  the  cord,  and  sometimes  tracts  of 
irregular  extent  and  position  are  involved,  without  connection  with 
the  membranes.  This  cause  is  one  of  especial  importance,  because  it 
often  co-operates  with  others,  and  determines  effects  that  may  seem 
to  be  independent.  Its  effect  is  met  with,  for  example,  in  many  cases 
of  chronic  myelitis  that  are  excited  by  injury  and  seem  to  be  due 
solely  to  the  excitant,  although  the  results  of  this  would  have  been 
trifling  and  transient  but  for  the  tendency  induced  by  the  preceding 
and  often  profoimd  effect  of  chronic  alcoholism  on  the  tissues.  It 
also  complicates  many  cases  of  multiple  neuritis  due  to  the  same 
cause,  in  which  the  effects  of  the  peripheral  lesion  obscure  to  a  large 
extent  those  of  the  central  mischief,  by  causing  symptoms  that  pre- 
vent the  recognition  of  the  latter,  since  its  consequences  are  lost,  as  it 
were,  in  those  of  the  damage  to  the  nerves. 

The  gouty  diathesis  is  another  cause  of  chronic  myelitis.  We  are 
only  beginning  to  realise  how  often  it  acts  on  many  organs  and  tissues, 
and  how  frequently  inflammation  is  thus  induced.  It  is  certain  that 
this  is  a  common  cause  of  inflammation  in  the  substance  of  organs 
and  in  mucous  membranes,  and  chronic  as  well  as  acute  myelitis  is 
among  its  effects.  Many  cases  are  due  to  this  influence,  and  it  is 
instructive  to  note  that,  like  chronic  alcohoHsm,  it  is  also  a  cause 
of  inflammation  of  the  peripheral  nerves.  The  disseminated  and 
progressive  forms  of  chronic  myehtis  are  especially  due  to  it,  and  the 
analogy  should  be  noted  which  these  present  to  the  irregular  forms 
of  perineuritis,  of  which  this  state  is  also  a  common  cause.  It  is 
probable  that  many  cases  of  chronic  myelitis  in  young  adults,  at 
present  mysterious  in  origin,  and  in  the  obstinately  progressive 
character  they  sometimes  manifest,  will  be  found  to  be  due  to  the 
influence  of  inherited  gout,  which  is  certainly  capable  of  causing 
analogous  inflammation  elsewhere  in  the  nervous  system, — as,  for 
instance,  in  the  optic  nerves. 

Another  cause  of  chronic  myelitis  is  constitutional  syphilis.  The 
forms  that  are  apparently  due  to  this  are  of  two  types.  (1)  A  diffuse 
interstitial  inflammation,  most  intense  at  the  periphery,  with  much 
new  formation  of  tissue  and  thickening  of  the  walls  of  the  vessels, 
both  processes  extending  into  the  cord  from  the  pi  a  mater,  in  which  a 
similar  damage  exists.  This  form  may  be  subchronic  in  course,  and 
presents  some  histological  correspondence  to  known  syphilitic  pro- 
cesses. (2)  A  focal  very  chronic  indurating  myelitis,  such  as  shown 
in  Fig.  106,  which  has  no  distinctively  syphilitic  feature,  may  occur  in 


OHEONIO  MYELITIS. 


381 


very  late  stages  of  the  constitutional  disease.  In  neither  form  does 
the  influence  of  treatment  afford  evidence  of  the  relation  to  syphilis. 
In  the  second,  sclerotic  variety  it  does  not  appear  that  treatment  has 
any  power  to  modify  the  morbid  process,  and  in  the  first  it  is  evident 
that  any  cicatricial  process  that  could  be  induced  would  only  maintain 
the  damage  to  the  nerve-elements,  which  would  suffer  further  from  the 
maintained  or  renewed  compression  under  the  prolonged  contraction 
of  the  enclosing:  tissue.    Yet  the  occurrence  of  these  forms  in  the 


Fia.  106. 


Fi&.  107. 


Fia.  106. — Chronic  myelitis.  (After  Charcot  and  Gombanlt.)  B,  section 
at  the  level  of  the  tliird  dorsal,  shows  hoth  the  posterior  columns  and  whole 
of  left  half  inflamed,  except  the  inner  part  of  the  anterior  column.  The 
affected  part  was  grey  and  uniform  in  aspect,  vascular,  and  firmer  than 
normal.  .  Pia  mater  and  arachnoid  thickened.  A,  section  from  cervical 
enlargement,  showing  secondary  degeneration  in  the  post.-med.  columns. 
Similar  foci  existed  in  the  pons,  &c.* 

FiO.  107. — Chronic  sclerotic  myelitis.  (After  Troisier.)  A,  posterior  view 
of  the  affected  part,  lower  dorsal  region.  The  diseased  areas  were  grey  in 
aspect,  and  are  indicated  by  shading.  B,  middle  of  lesion,  showing  exten- 
sive damage  ;  C,  2|  cm.  higher  up,  slighter  damage ;  D,  cervical  region 
ascending  degeneration,  post.-med.  cols.,  and  a  spot  of  disease  in  lateral 
column  (probably  part  of  the  ascending  ant.-lat.  tract,  secondarily  dege« 
nerated).    E,  lower  part  of  lesion.     (See  next  figure.)t 


*  The  patient  had  had  syphilis  twenty-one  years  before.  The  spinal  symptomt 
slowly  developed  during  fifteen  months,  and  consisted  in  paralysis  with  hyper- 
sesthesia  on  the  left  side,  with  loss  of  sensibility  without  paralysis  in  the  right,  and 
a  band  of  anaesthesia  to  pain  around  the  thorax  at  the  level  of  the  lesion,  but  the 
mental  dnlness  of  the  patient  prevented  accurate  observation  during  the  last  few 
months  of  her  life.  (Charcot  and  Gombanlt,  'Arch,  de  Phys.,'  1873,  vol.  vi, 
p.  143.) 

t  The  patient  was  a  woman  forty  years  of  age.  The  symptoms  slowly  developed 
during  the  three  years  before  death,  and  consisted  of  weakness  with  rigidity  in  the 
left  leg,  and  impairment  of  sensibility  in  the  right,  first  of  the  temperature  sense 
and  afterwards  of  touch.  (No  observations  were  made  on  sensibility  for  the  last 
fifteen  months  of  the  patient's  life,  during  which  the  lesion  was  slowly  progressing.) 
(Troisier.  *  Arch,  de  Physiologic,*  1873,  vol.  y,  p.  716,  Case  2.) 


882  SPINAL    COED. 

subjects  of  syi^liilis,  tlie  absence  of  otber  causes,  and  tbe  circamstances 
that  the  negative  facts  are  not  without  analogies,  make  this  causation 
on  the  whole  the  probable  one. 

PATHOLoaicAii  Anatomy. — The  morbid  appearances  vary  much  in 
the  different  forms.  In  most  cases  the  disease  is  revealed  to  the 
naked  eye  by  a  change  in  the  aspect  of  the  affected  part,  in  which  the 
white  substance  is  grey  in  tint,  and  the  grey  matter  darker  than 
normal,  while  both  are  reduced  in  size  in  cases  of  long  duration,  so 
that  the  whole  cord  may  be  lessened  at  the  affected  part,  and  its 
shape  changed  when  the  disease  does  not  involve  its  entire  thickness. 
But  in  recent  cases  the  alteration  in  tint  may  be  the  most  conspicuous 
change,  and  may  be  visible  on  the  surface,  even  through  the  pia 
mater ;  this  may  be  thickened  at  the  spot  if  the  disease  reaches  the 
surface.  The  consistence  of  the  affected  area  may  be  lessened  or 
increased.  Rarely,  in  recent  cases,  there  is  a  slight  increase  in  the 
size  of  the  affected  part.  In  some  recorded  cases  in  which  a  great 
increase  in  size  has  been  described,  it  is  probable  that  an  infiltrating 
glioma  was  mistaken  for  chronic  inflammation.  When  there  is  one 
considerable  focus  of  disease  there  may  be  other  slighter  foci  in  the 
neighbourhood.  In  what  is  called  the  "  diffuse "  form  the  diseased 
areas  are  scattered  through  a  considerable  extent  of  the  cord ;  they 
may  blend  into  a  lesion  that  is  truly  diffuse,  or  may  remain,  in  point 
of  fact,  disseminated,  but  be  so  placed  that  their  effects  blend  in  what 
may  be  termed  a  diffuse  influence.  Thus  it  may  be  found,  post  mortem, 
that  there  are  numerous  foci  of  disease  when  nothing  in  the  character 
of  the  symptoms  prepared  us  for  a  disseminated  lesion. 

It  is  probable,  indeed,  that  this  is  the  rule  in  the  chronic  form,  and 
an  actually  diffuse  extensive  inflammation  is  much  more  rare  than  is 
suggested  by  the  symptoms  of  the  disease.  In  the  secondary  form 
the  inflammation  is  usually  confined  to  the  neighbourhood  of  the 
morbid  process  which  causes  it.  That  which  is  secondary  to  menin- 
gitis is  most  intense  in  the  peripheral  part  of  the  cord,  but  sometimes 
extends  deeply,  especially  when  the  cause  of  the  meningitis  is  one, 
such  as  alcoholism,  capable  also  of  giving  rise  to  myelitis.  In  this 
condition  almost  the  whole  thickness  of  the  cord  may  be  involved  (of. 
A  and  B,  Fig.  109).  Now  and  then,  when  there  is  no  primary  menin- 
gitis, the  superficial  layers  of  the  cord  are  chiefly  damaged,  a  form  that 
has  been  termed  chronic  annular  myelitis  or  annular  sclerosis,  because 
the  affection  extends  like  a  ring  around  the  cord.  This  form  of  lesion 
is,  however,  as  we  have  seen,  sometimes  merely  an  ascending  degene- 
ration of  fibres  that  lie  near  the  surface,  and  degenerate  above  the 
primary  lesion. 

In  chronic  myelitis,  according  to  the  limited  use  of  the  term 
already  explained,  the  microscope  shows  that  the  inflammation  is 
primarily  interstitial.  The  affected  areas  always  stain  deeply  with 
carmine  in  consequence  of  the  great  increase  in  their  connective-tissue 
elements,  and  may  be  occupied  by  a  dense  fibrous  reticulum,  in  which 


CHBONIO    MYELITIS. 


383 


few  nerve-elements  can  be  discovered,  as  in  Fig.  108.  In  less  affected 
parts  of  the  wMte  columns  there  is  an  irregular  increase  of  the 
interstitial  tissue,  partly  fibrillated,  partly  amorphous,  or  studded  with 
nuclei  (Fig.  108,  upper  small  figure)  ;  while  in  other  parts  it  may  contain 
many  nucleated  cells,  oval,  fusiform,  or  stellate.  The  large  stellate 
"  spider  cells "  are  sometimes  very  conspicuous,  and  interstitial 
fibrous  tracts  may  be  traced  to  their  ramifications. 


r^rr^ 


Fig.  108.— Chronic  sclerotic  myelitis,  same  case  as  Fig.  107;  section  near  B, 
reversed.  Almost  the  whole  left  lialf  of  the  cord  (right  in  the  figure)  is 
changed  into  a  dense  mass  of  shrunken  connective  tissue,  and  the  right  half 
is  being  invaded  in  many  parts  by  the  same  process.  The  upper  small  figure 
is  from  the  posterior  column,  showing  a  thickened  vessel  surrounded  by  nucleated 
tissue,  among  which  lie  nerve- fibres,  many  of  them  much  smaller  than  normal, 
and  some  with  swollen  axis-c.\lindcrs.  The  lower  small  figure  is  fi-om  the  right 
anterior  column  (left  in  figure);  the  pia  mater  is  thickened  and  contains  many 
nuclear  elemems  j  from  it  thick  trabeculae  extend  into  the  white  substance  and 
enclose  spaces,  from  many  of  which  the  nerve-fibres  have  perished.* 

The  nerve-fibres  waste  before  the  interstitial  process,  or  undergo 
destructive  changes  from  the  beginning.  They  may  at  first  present 
the  change  by  which  their  myelin  stains  more  deeply  than  normal  ; 
afterwards  they  become  narrowed,  and  the  axis-cyhnder  is  often 
irregularly  enlarged.  Ultimately  many  or  all  the  fibres  disappear 
from  the  affected  area.  Here  and  there  the  destruction  of  fibres  may 
be  out  of  proportion  to  the  increase  in  the  interstitial  tissue,  so  that 
thickened  and  often  granular  trabeculse  enclose  empty  spaces.  This 
is  seen  in  the  lower  small  illustration  in  Fig.  108 ;  it  is  apparently  the 
residt  of  the  participation  of  the  nerve-fibres  in  the  inflammatory 
process  during  a  more  general  stage,  in  which  all  the  elements  of  the 
tissue  were  involved.  In  the  recent  state,  products  of  degeneration, 
granule   corpuscles,    &c.,    may   be  found  abundantly  in  the  altered 

*  From  a  section  by  Prof.  Pierrot,  kindly  lent  me  by  Dr.  Dreschfeld. 


384  SPINAL   CORD. 

regions.  The  -walls  of  tlie  vessels  are  thickened,  and  by  this 
thickening  the  cavities  of  the  arteries  may  be  ultimately  lessened; 
those  of  smaller  size  may  even  he  closed.  Not  only  is  the 
wall  of  the  arteries  thickened,  but  around  them  the  increase  of  inter- 
stitial tissue  is  often  greater  than  elsewhere,  so  that  the  appearance  is 
as  if  it  had  spread  thence  into  the  adjacent  structures.  Undue  signi- 
ficance has  been  attached  to  this  condition,  especially  in  old  patients 
■with  arterial  degeneration;  it  has  been  regarded  as  evidence  that 
arterial  disease  sets  up  the  myelitis,  but  it  is  a  common  appearance  in 
all  forms  of  chronic  myelitis,  and  also  in  the  later  stages  of  acute 
inflammation.  It  is  probably  due  to  the  greater  activity  of  the  process 
in  the  immediate  vicinity  of  the  vessels,  and  to  the  share  taken  by 
escaped  leucocytes  in  the  formation  of  new  interstitial  tissue.  The 
peripheral  arteries,  moreover,  occupy  tracts  of  neuroglial  and  fibrous 
tissue  proceeding  from  the  pia  mater,  and  the  increase  of  interstitial 
tissue  is  connected  with  these ;  some  of  it  is,  on  this  account,  found 
in  the  vicinity  of  the  arteries. 

In  the  grey  substance  there  is  a  similar  increase  of  connective- 
tissue  elements,  so  that  the  altered  part  takes  a  deeper  stain  with 
carmine  than  the  normal  grey  substance,  and  has  a  denser  structure, 
from  the  greater  amount  of  supporting  tissue.  The  nerve-elements 
become  atrophied ;  the  ganglion-cells,  at  first  swollen  and  rounder, 
afterwards  shrink,  until  ultimately  they  may  be  reduced  to  small 
angular  bodies,  and  they  even  may  disappear.  The  nuclear  corpuscles 
about  the  central  canal  are  often  increased  in  quantity,  and  the  canal 
obliterated,  but  this  condition  is  so  common  in  otherwise  normal  cords 
that  no  importance  can  be  attached  to  it.  A  considerable  increase  of 
tissue  about  the  central  canal,  closing  it  below  and  causing  it  to  be 
dilated  above  (Fig.  109,  "  syringomyelia "),  has  been  ascribed  to 
chronic  inflammation  around  the  canal,  but  the  abnormal  tissue  is 
probably  always  of  gliomatous  nature,  consisting  of  a  development 
of  tissue  that  is  in  congenital  excess,  owing  to  an  arrest  of  develop- 
ment. 

The  pia  mater  is  greatly  thickened  over  the  inflamed  parts,  as 
already  stated,  and  in  some  cases  the  affection  of  the  membrane  is 
general,  and  that  of  the  cord  most  intense  beneath  it— a  condition 
termed  "  chronic  meningo-myelitis."  It  is  this  form  that  is  shown  in 
Fig.  109.  The  tracts  of  new  tissue  that  pass  into  the  cord  are  dense 
and  wide.  As  already  mentioned,  this  change  may  be  accompanied 
by  a  deeper  and  sometimes  a  more  diffuse  inflammation,  as  in  a.  Fig. 
109,  in  which  the  affection  of  the  cord  is  greater  and  that  of  the  mem- 
branes less  than  ia  b.  The  chronic  myelitis  that  occurs  in  syphilitic 
subjects  may  present  no  specific  characters.  In  some  cases,  however, 
the  cell-formation  has  been  abundant,  and  has  been  associated  with  a 
tendency  to  fatty  degeneration  in  the  older  portions  of  the  lesion,  and 
with  a  similar  degenerating  hyperplastic  inflammation  of  the  pia 
mater.     The  condition  is,  however,  clearly  one  of  chronic  inflamma- 


CHUONIO  MYELITIS. 


885 


tion,  and  not  of  a  sjpliilitic  growth.*    Nevertheless  an  actual  growth 
does  sometimes  co-exist  with  a  focus  of  syphilitic  myelitis ;  it  is  pro- 


Flff.  109.— Chronic  menirgo-iryelitis,  due  to  alcoholism.  Syring'O-myelia, 
CongenitHl,  with  central  gliomatosis.  A,  lowest  part  of  cervic;il  enlai^e- 
ment;  pia  mater  thickened,  and  from  it  tracts  of  tissue  extend  into  the 
cord.  The  greater  part  of  the  section  is  the  seat  of  a  diffuse  myelitis, 
in  which  the  nerve-fibies  could  be  seen  with  d ifficulty,  and  were  sepa« 
rated  by  inflammatory  products.  The  grey  matter  was  also  affected, 
and  the  nerve-cells  much  changed.  The  clear  spaces  in  the  right  ant. 
coma  appeared  occupied  by  products  of  degeneration.  Central  canal 
greatly  dilated  and  surrounded  by  new  growth,  which  in  B  (lumbar  sec- 
tion) has  obliterated  the  canal  and  thus  cau-ed  its  dilatation  above.  In 
this  section  the  myelitis  is  chiefly  peripheral,  and  the  grey  matter  has, 
for  the  most  part,  escaped. 

bable  that  in  such  cases  the  growth  is  usually  the  primary  change. 
This  may  be  distinguished  by  the  indications  of  compression  and 
displacement  of  the  adjacent  elements  of  the  cord,  a  condition  which 
shows  that  there  is  an  actual  new  formation. 

The  more  recent  the  inflammation,  the  larger  is  the  proportion  of 
cells  and  nuclei  in  the  interstitial  tissue;  the  longer  it  has  existed, 

*  See  Moxon,  '  Guy's  Hosp.  Reports,'  1871,  and  Charcot  and  Gombault,  *  Arch» 
de  Physiologie,'  1873. 

VOL.  I.  25 


886  SPINAL    CORD. 

the  greater  is  tlie  amount  of  fibrillation  it  presents.  The  very  chronic 
indurating  forms  are  sometimes  called  "  sclerotic  myelitis,"  or  "  focal 
sclerosis  of  the  cord."  From  the  various  areas  of  inflammation  secon- 
dary degenerations  proceed,  ascending  or  descending,  and  these  may 
complicate  the  aspect  of  the  disease- — a  fact  which  has  not  always  been 
sufficiently  considered  in  interpreting  the  morbid  appearances. 

Symptoms. — The  symptoms  of  chronic  myelitis  vary  much  according 
to  the  character  of  the  morbid  process.  For  the  most  part  they  cor- 
respond in  character  to  those  that  are  produced  by  acute  inflamma- 
tion, differing,  however,  in  their  mode  of  onset.  It  is  therefore  not 
necessary  to  repeat  them  in  detail.  Different  cases  may  manifest 
almost  every  symptom  that  can  be  produced  by  disease  of  the  spinal 
cord.  There  may  be  various  motor  and  sensory  paralyses,  contrac- 
txires,  and  even  muscular  atrophy,  but  their  common  feature  is  their 
gradual,  and  often  successive  development.  In  the  most  common  form, 
focal  myelitis  of  the  dorsal  region,  partial  or  transverse,  the  symptoms 
are  paraplegic.  They  differ  but  little  whether  there  is  a  single  area 
of  disease,  or  whether  several  foci  near  together  implicate  adjacent 
structures.  Motor  power  is  usually  impaired  far  more  than  sensi- 
bility. Subjective  sensations  are  often  prominent  and  early  symptoms, 
whether  there  is  anaesthesia  or  not, — tingling,  "  pins  and  needles  "  in 
the  legs,  a  sensation  as  if  fur  or  wool  covered  the  skin,  sometimes  with 
dull  pain  in  the  legs  and  back,  especially  after  exertion,  and  commonly 
also  with  a  well-marked  girdle  pain  at  the  level  of  the  chief  lesion. 
Some  loss  of  sensibility,  partial  in  kind  or  in  seat,  will  often  be  found 
if  carefully  searched  for,  but  there  is  not  often  absolute  loss  of  sensa- 
tion except  in  the  rare  cases  of  intense  local  sclerotic  myelitis,  by 
which  conduction  may  be  arrested  as  completely  as  by  a  growth. 
The  onset  of  the  motor  weakness  is  gradual ;  many  months  may  pass 
before  the  power  of  walking  is  considerably  impaired.  As  the  legs 
get  weak,  excess  of  reflex  action  is  developed  (unless  the  reflex  struc- 
tures are  damaged).  The  knee-jerk  is  increased ;  foot-clonus  occurs 
when  the  legs  are  in  certain  positions ;  and  a  tendency  to  spasm 
gradually  develops,  and  increases  to  the  condition  typical  of  "  spastic 
paraplegia."  The  sphincters  frequently  share  the  impairment  of 
voluntary  power.  Co-ordination  of  movement  is  sometimes  affected, 
usually  manifested  as  general  tmsteadiness,  and  defect  of  equilibrium, 
rather  than  as  pure  ataxy. 

The  symptoms  may  come  on  simultaneously  in  the  two  legs,  or  one 
may  be  affected  before  the  other,  and  occasionally  the  paralysis  reaches 
a  high  degree  in  one  leg  while  the  other  still  possesses  fair  strength. 
When  one  half  of  the  cord  is  affected  almost  alone  (as  in  the  lesion 
shown  in  Fig.  109),  there  may  even  be  a  crossed  paralysis  of  motion 
.and  sensation. 

Chronic  polio-myelitis  is  often  regarded  as  a  distinct  variety,  but 
much  confusion  has  arisen  from  the  fact.  If  chronic  myehtis  de- 
velops in  the  cervical  and  lumbar  enlargement,  muscular  wasting  is 


CHRONIC   MYELITIS.  387 

usually  added  to  tlie  other  symptoms.  Similar  atropliy  occurs  also 
in  the  widely  diffused  form  of  chronic  myelitis,  in  which  almost  all 
parts  of  the  cord  suffer,  and  it  may,  indeed,  be  the  most  conspicuous 
symptom.  It  is  always  irregular  in  distribution  in  these  cases,  and  it 
may  be  combined  with  other  symptoms,  palsy  and  anaesthesia,  also 
irregular  in  extent  and  seat.  The  muscular  wasting  varies  in  the 
rapidity  of  the  development,  according  to  the  character  of  the  inflam- 
mation, and  there  are  corresponding  variations  in  the  relations  to  it  of 
the  loss  of  power  in  the  muscles,  and  also  in  the  electrical  reactions 
they  present.  In  very  chronic  cases  there  is  a  slow  failure  of  electrical 
irritability  to  both  currents  ;  in  others  of  more  rapid  course  the  voltaic 
irritability  persists  longer  than  the  faradic  irritability,  and  there 
may  be  a  distinct  degenerative  reaction.  The  symptoms  may  com- 
mence in  the  arms  or  in  the  legs,  but  they  ultimately  become  general 
in  most  cases,  and,  according  to  the  place  of  their  commencement, 
may  seem  to  have  an  ascending  or  descending  course.  Occasionally 
the  disease  begins  in  the  dorsal  region,  and  after  a  time,  perhaps 
years,  it  spreads  to  the  enlargements,  and  the  muscular  wasting  is 
added. 

The  course  of  chronic  myelitis  is  usually  slow  and  progressive.  The 
symptoms  often  increase  so  gradually  as  only  to  attain  a  considerable 
degree  at  the  end  of  two  or  three  years.  At  any  stage  the  progress 
of  the  disease  may  cease.  The  chronic  course  may  be  varied  by 
occasional  more  rapid  increase  of  the  symptoms,  due  to  subacute  or 
even  acute  processes  in  the  diseased  parts.  Every  degree  of  chronicity 
is  met  with,  and  subchronic  cases,  in  which  the  symptoms  develop  in 
the  course  of  a  few  months,  effect  a  gradation  to  the  acute  and  sub- 
acute forms  of  myelitis.  The  duration  of  the  disease  varies  from  six 
months  to  ten  years  or  more.  Thus  in  one  case  the  symptoms  slowly 
increased  during  about  seven  years,  and  then  became  stationary,  and 
the  patient  died  twenty-three  years  after  the  onset. 

Diagnosis. — The  diagnosis  of  chronic  myelitis  rests  on  the  slow  deve- 
lopment of  symptoms  indicating  a  random  process  in  the  spinal  cord, 
i.  e.  a  process  which  damages  irregularly  structures  of  various  functions, 
and  thus  cannot  be  looked  on  as  a  "  system  disease."  The  maladies 
from  which  it  has  to  be  distinguished  differ  according  to  the  seat  and 
character  of  the  inflammation.  Dorsal  transverse  or  focal  myelitis 
may  be  confounded  with  compression  of  the  cord,  with  a  tumour  in  it, 
and  with  primary  lateral  sclerosis.  The  distinction  from  pressure 
rests  on  the  absence  of  a  cause  of  compression,  such  as  bone  disease, 
— especially  the  absence  of  the  signs  of  a  morbid  process  outside  the 
cord,  preceding  those  of  damage  to  the  cord  itself,  e.  g.  the  pains 
that  indicate  irritation  of  nerve-roots.  A  tumour  within  the  spinal 
cord  also  causes,  in  most  cases,  more  irritation  of  the  root-fibres  than 
does  chronic  myelitis.  If  myelitis  involves  one  half  of  the  cord 
much  more  than  the  other,  the  symptoms  may  closely  resemble  those 
of  an  intra-medullary  growth.     But  a  tumour  never  causes  much 


388  SPINAL   COED. 

damage  to  one  lialf  of  the  cord  witliout  interfering  considerably  with 
the  functions  of  the  other  half,  and  the  symptoms  in  chronic  myelitis 
may  be  strictly  unilateral.  The  distinction  from  primary  spastic 
paraplegia,  the  so-called  "  primary  lateral  sclerosis,"  is  often  one  of 
some  difficulty.  The  motor  state  of  the  legs  may  be  identical  in  the  two 
diseases  ;  in  each  there  is  the  same  extensor  spasm,  and  in  each  there 
is  a  slow,  gradual,  and  apparently  simultaneous  onset  of  the  weakness 
and  spasm.  The  distinction  is  that  in  primary  spastic  paraplegia  the 
symptoms  are  purely  motor;  there  is  no  indication  that  the  lesion 
extends  beyond  the  motor  structtwes.  In  chronic  myehtis,  on  the 
other  hand,  there  is  such  indication,  either  by  the  impairment  of 
sensation,  or  by  the  presence  of  a  girdle  pain.  This  distinction  is 
sufficient  in  the  majority  of  cases.  The  distinction  from  insular 
sclerosis  is  in  many  cases  difficult.  By  Oppenheim  and  others, 
hideed,  it  is  asserted  that  most  cases  described  as  chronic  myelitis 
are  really  cases  of  disseminated  sclerosis.  The  presence  of  cerebral  or 
bulbar  symptoms  would  naturally  suggest  such  a  diagnosis  in  a  given 
case.  But  in  insular  sclerosis  any  very  obvious  affection  of  sensation 
is  unusual. 

The  form  that  involves  the  grey  matter  has  to  be  distinguished 
chiefly  from  pachymeningitis  and  degenerative  muscular  atrophy. 
In  pachymeningitis  the  muscular  wasting  may  be  similar,  but 
anaesthesia  is  usually  much  more  considerable  in  range  and  in  degree, 
and  there  is  more  pain  in  the  limbs.  If  there  are  similar  symptoms 
in  both  arms  and  legs,  myelitis  is  far  more  probable  than  pachy- 
meniugitis,  since  the  chronic  inflammation  of  the  membranes  is  less 
extensive  than  that  of  the  cord.  From  progressive  muscular  atrophy, 
the  chief  difference  is  in  the  random  distribution  of  the  wasting,  the 
absence  of  symmetry,  and  indications  of  irregular  damage  to  other 
structures  in.  the  cord,  as  the  occurrence  of  pain  and  of  loss  of 
sensation.  But  it  must  be  remembered  that,  by  some  authorities, 
most  cases  of  chronic  spinal  muscular  atrophy  are  regarded  as  due  to 
chronic  myelitis. 

Eor  the  distinction  from  other  less  common  diseases,  the  reader 
must  be  referred  to  the  description  of  them.  Like  all  other  organic 
diseases,  cases  without  wasting  of  the  muscles,  occurring  in  females, 
are  often  regarded  as  "  hysterical  paraplegia ;  "  the  distinction  rests 
on  the  points  already  described.  It  is,  however,  one  of  the  diseases 
in  which  the  least  excusable  er.  ors  m  diagnosis  occur,  and  in  which 
their  effects  are  often  the  most  disastrous. 

Prognosis. — Chronic  myelitis  is  a  very  grave  disease,  on  account  of 
the  intractability  of  the  morbid  process,  its  frequently  progressive 
tendency,  and  the  persistence  of  damage  which  is  slowly  produced. 
But  cases  vary  widely,  especially  in  the  two  former  features,  and  it  is 
a  disease  in  which  general  prognostic  rules  can  scarcely  be  formulated. 
The  observed  tendency  of  an  individual  case  is  alone  a  trustworthy 
guide.     Arrest  of  the  disease  is  often  obtained,  but  actual  recovery 


CHEONIO    MYELITIS.  389 

is  rare.  The  prognosis  is  little  affected  by  the  seat  or  extent  of  tLe 
process,  except  that  the  implication  of  the  grey  matter  of  the  enlarge- 
ments is  generally  an  unfavorable  indication.  The  severity  of  the 
lesion,  as  shown  by  the  degree  of  the  symptoms,  is  significant  chiefly 
in  cases  of  long  duration,  and  is  subordinate  to  the  course  and  mode 
of  onset.  If  it  can  be  arrested,  the  prospect  of  improvement  is  better 
in  proportion  to  the  slowness  with  which  the  disease  has  developed  and 
the  shortness  of  the  time  the  symptoms  have  lasted.  In  general,  more- 
over, the  prospect  is  better,  the  fewer  the  foci  of  inflammation,  in  males 
than  in  females,  and  in  cases  of  uniform  than  in  those  of  recurrent  or 
relapsing  course.  Preceding  syphilis  does  not  materially  modify  the 
pi'Ognosis ;  hence  the  great  importance  of  the  diagnosis  from  syphilitic 
-growth,  in  which  suitable  treatment  has  a  most  certain  effect.  The 
ultimate  danger  to  life  is  least  in  focal  myelitis  in  the  dorsal  region  ; 
it  is  greatest  in  the  disseminated  form,  especially  in  the  less  chronic 
cases  with  a  tendency  to  sloughing  of  the  skin,  and  in  the  more 
chronic  forms  with  muscular  wasting.  The  indications  drawn  from 
the  invasion  of  the  respiratory  muscles,  and  from  the  presence  of  any 
of  the  complications  that  so  often  terminate  life,  are  the  same  as  in 
other  diseases  of  the  cord. 

Tkeatment. — The  first  and  most  important  measui-e  is  the  improve- 
ment of  the  general  health,  by  rest,  change  of  air,  and  tonics.  All 
■causes  of  physical  and  mental  depression  must,  as  far  as  possible,  be 
removed.  Over-exertion,  and  even  fatigue,  should  be  avoided,  and 
the  patient  should  be  kept,  as  far  as  possible,  from  exposure  to  cold. 
Absolute  rest  for  a  short  time  is  often  useful  at  the  outset  of  the 
treatment,  especially  when  there  has  been  a  somewhat  rapid  develop- 
ment of  symptoms.  In  cases  of  purely  chronic  course  absolute  rest 
should  not  be  maintained  for  more  than  a  few  weeks,  or  the  patient 
may  find  it  hard  to  regain  his  former  muscular  power.  Counter- 
irritation  at  the  situation  of  the  disease  is  often  useful,  and  most  so  in 
cases  in  which  there  is  spinal  pain  or  tenderness.  Repeated  siaapisms 
or  blisters  may  be  employed,  but  a  mild  form  of  the  actual  cautery  is 
on  the  whole  the  most  useful.  A  superficial  bum  or  even  slight 
vesication  is  sufficient  on  each  side  of  the  spine  opposite  the  affected 
part,  and  it  is  best  effected  by  means  of  the  Paquelin  cautery.  It 
should  be  repeated  as  soon  as  the  skin  has  healed,  and  if  linear 
cauterisation  is  adopted  the  fresh  line  can  be  made  beside  the  old  one. 
A  hot  douche  to  the  back,  at  a  temperatvire  of  103°  or  104"  F.,  applied 
daily,  has  been  strongly  recommended  by  Brown-Sequard.  Warm 
brine  baths,  and  various  thermal  mineral  waters,  have  been  also  said 
to  do  good.  A  sea  voyage  is  often  of  service,  combiaing  as  it  does 
the  maximum  of  fresh  air  with  the  minimum  necessity  for  exertion. 
Drugs,  Tinfortunately,  often  fail  to  influence  the  morbid  process,  but 
nevertheless  they  are  occasionally  useful,  so  as  distiactly  to  justify  their 
careful  trial  in  most  cases ;  and  it  is  important  to  remember  that  those 
that  are  useless  in  one  stage  or  period  of  the  disease  may  yet  be  of 


S90  SPINAL    CORD. 

unquestionable  service  at  another  stage.  Tonics,  as  quinine  and  iron» 
should  be  given  if  indicated.  Most  of  the  measures  recommended, 
and  precautions  advised,  in  the  treatment  of  acute  myelitis  are 
needed  in  the  chronic  form,  and  it  is  therefore  superfluous  to  repeat 
them.  Those  drugs  that  most  deserve  a  trial  are  arsenic,  small 
doses  of  mercury  (such  as  -^  gr.  of  the  red  iodide),  and  iodide  of 
iron.  Energetic  mercurial  treatment  rarely  does  good,  even  when  the 
patient  has  had  syphilis,  and  sometimes  it  does  harm.  ■  Iodide  of 
potassium  seems  to  have  little  influence  on  the  disease.  Nitrate 
of  silver,  ergot,  and  phosphorus  have  been  recommended.  Strychnia 
is  chiefly  useful  in  cases  in  which  there  is  muscular  wasting,  but  is 
of  far  less  value  than  in  the  degenerative  muscular  atrophy.  The 
treatment  of  other  symptoms  is  that  suitable  in  primary  spastic 
paraplegia  or  progressive  muscular  atrophy,  and  described  in  the 
account  of  those  diseases. 


COMPRESSION  OF  THE   SPINAL  CORD. 

Compression  of  the  spinal  cord  is  a  common  consequence  of  various 
morbid  processes.  Inflammation  is  almost  always  excited  by  the 
pressure,  and  intea-ference  with  function  occurs,  partly  from  the 
pressure  and  partly  from  the  resulting  myelitis.  The  symptoms  pro- 
duced have,  in  different  cases,  many  characters  in  common,  although 
they  vary  according  to  the  mechanism  of  the  compression  and  the 
acuteness  of  the  inflammation.  It  is  only  when  there  are  indications 
that  an  acute  myelitis  has  damaged  the  nerve-elements  that  we  are 
justified  in  regarding  the  interference  with  function  as  an  effect  of 
the  inflammation. 

Causes. — The  morbid  processes  that  may  compress  the  cord  are 
those  that  involve  an  encroachment  on,  or  occupy  part  of,  the  vertebral 
canal.  The  chief  are  the  following: — Disease  of  the  bones  of  the 
spinal  column,  especially  caries ;  growths  in  the  spine ;  aneurism 
eroding  the  bones  and  then  compressing  the  cord;  growths  in  the 
membranes;  thickening  of  the  dura  mater.  These  processes  have 
usually  only  a  small  vertical  extent,  and  the  pressure  they  exert  rarely 
extends  over  more  than  a  few  inches. 

Pathological  Anatomy. — The  cord  usually  presents  evidence  of 
the  compression  it  has  endured  in  considerable  narrowing  at  the  spot, 
where  it  may  be  indented  and  flattened,  or  cyhndrical.  Sometimes 
the  reduction  in  size  is  extreme ;  for  an  inch  or  so  the  cord  may  be 
reduced  to  one  third  of  its  normal  diameter,  and  it  has  even  been 
found  no  thicker  than  a  crow-quill.      An  example   of  flattening  is 


COMPRESSION. 


89] 


/' 


C 


•hewn  in  Fig.  110.  On  the  otlier  hand, 
there  is  sometimes  very  little  narrowing 
to  be  discerned.  At  the  compressed  part 
the  cord  is  usually  red  in  tint  if  the  com- 
pressio  i  is  recent,  but  grey  if  it  has  lasted 
for  some  time  ;  its  consistence  is  lessened  in 
early  cases,  and  increased  in  those  of  long 
duration.  The  change  in  colour  and  con- 
sistence is  due  to  the  inflammation  of  the 
substance  of  the  cord  which  always  results 
from  pressure,  and  may  often  be  traced  for 
some  distance  above  and  below  the  com- 
pressed part.  When  there  is  much  com- 
pression there  is  always  much  inflammation, 
but  considerable  myelitis  may  occur  when 
the  amount  of  compression  is  slight.  The 
inflammation  may  be  chronic  and  slow, 
developing  in  proportion  to  the  pressui-e,  or 
it  may  be  subacute  or  acute,  even  when  the 
pressure  is  gradual.  The  signs  of  inflam- 
mation are  very  distinct  on  microscopical 
examination,  and  resemble  those  in  other 
forms  of  myelitis.  There  is  a  general  in- 
crease in  the  interstitial  tis-ue ;  in  this,  at 
first,  various  cell-forms  may  be  found,  but 
it  ultimately  presents  the  appearance  of  a 
dense  reticulum.  The  nerve-elements  un- 
dergo degeneration,  and  abimdant  masses 
of  myelin,  granule  corpuscles,  and  corpora 
amjlacea  are  visible  in  the  fresh  state  or 
in  glycerine  preparations  of  the  hardened 
cord  (Fig.  Ill,  c).  Many  nerve-fibres  per- 
sist, however,  with  a  narrowed  sheath  of 


Fia.  110. — Compression  of  the  spinal  cord  and  pres- 
sure-myelitis, in  a  CHse  of  caries  of  tlie  spine.  D, 
mid-dorsal  region,  m  ar  the  chief  point  of  greatest 
compression;  the  cord  is  narrowed  from  before 
backwards,  and  is  uniformly  damaged  by  inflsim- 
mation,  so  that  the  gr.  y  sub.-tance  can  ? cavc'  ly  be 
distinguished.  C,  1^  inches  higher  up,  sliows  a 
slighter  degree  of  myelitis,  still  extending  through 
the  whole  thickness  of  the  cord.  B,  first  dorsul;  the  myelitis  is  much  slighter 
and  the  chief  disease  is  in  the  posterior  columns,  in  which  ascending  degeneration 
occupies  the  post.-median  columns  and  extends  outwards  into  the  post.-external 
columns.  E,  1^  inches  below  the  point  of  greatest  pressure ;  infl-.immation  still 
extending  througli  all  the  elements  of  the  cord.  F,  2  inches  lower  down,  shows 
only  descending  degeneration  in  the  pyramidal  tracts,  anterior  and  lateral,  and 
also  the  "comma-shaped"  descending  degeneration  in  the  anterior  part  of  the 
post.-external  column.  In  G,  at  the  lowest  part  of  the  dorsal  region,  the  comma- 
shaped  degeneration  has  ceased,  and  only  that  of  the  pyramidal  tracts  remains. 
(From  preparations  by  Dr.  F.  G.  Penrose.) 


892 


SPINAL    (JORD. 


myelin,  and  it  is  probable  tbat  these  regain  tbe  power  of  conducting, 
in  spite  of  tbe  persistence  of  considerable,  and  even  dense  sclerosis  in 


::?s^r 


«?• 


:'*-;"   '^'o 


Pia.  111. — Pressure-myelitis;  portions  of  the  section  D  in  Fig.  110  more 
highly  magnified.  A,  from  the  grey  matter,  numerous  angular  and 
fusiform  cells;  vessels  with  walls  thickened  by  a  growth  of  cells,  nar. 
rowing  the  cavity,  which  in  some  is  obliterated.  B,  from  the  white 
columns  ;  thickening  of  the  interstitial  tissue,  nerve-fibres  in  part 
destroyed,  in  part  narrowed  J  a  vessel  with  thickened  walls.  C,  glycerine 
preparation  from  white  substance ;  abundant  products  of  degeneration 
of  the  nerve-fibres,  in  part  aggregated  into  granule  masses.  D,  section 
of  a  nerve-root  passing  by  compressed  area ;  increase  of  interstitial 
tissue,  many  nerve-fibres  narrowed,  some  with  swollen  axis-cylinders. 

the  part.  In  extreme  cases  all  the  fibres  seem  to  be  destroyed  at  the 
point  of  chief  compression,  but  there  is  never  an  actual  division  of  the 
cord  itself.  The  grey  matter  can  scarcely  be  distinguished  from  the 
white,  even  with  the  microscope,  and  the  ganglion-cells  become 
shrunken  and  atrophied.  The  walls  of  the  small  vessels  are  often 
thickened  by  spindle-cells,  disposed  more  or  less  concentrically  to  the 
cavity,  which  is  encroached  upon  and  may  be  obliterated  (Fig.  Ill,  a,  b), 
a  process  that  must  add  to  the  degree  of  damage  to  the  cord.  The 
signs  of  inflammation  gradually  lessen  above  and  below  the  compressed 
part,  but  often  extend  for  some  inches  in  each  direction.  Beyond  its 
limits  the  usual  ascending  and  descending  degenerations  may  be 
traced  (Fig.  110).  The  nerve-roots  passing  by  the  seat  of  compression 
suffer  from  the  pressure,  and  from  interstitial  inflammation  excited 
in  them.  They  are  usually  grey  in  tint  and  ultimately  waste,  and  may 
even  be  reduced  to  fibrous  threads.  The  microscope  shows  increase  of 
the  interstitial  tissue,  wasting  of  many  nerve-fibres  (Fig.  Ill,  d),  and 
enlargement  of  some  axis-cyhnders. 


COMPEKSSION.  893 

Symptoms. — The  effects  vary  much,  according  to  the  degree  of 
pressure,  its  rapidity,  its  direction,  the  amount  and  character  of  the 
inflammation  produced,  the  amount  of  damage  to  the  nerve-roots, 
and  the  position  of  the  disease.  The  symptoms  in  most  cases  enable 
the  fact  of  slow  compression  to  be  inferred,  even  when  there  is  no 
indication  of  the  cause.  They  are  of  two  classes :  (1)  interference 
with  the  function  of  the  nerve-roots  at  the  level  of  the  morbid  process ; 
(2)  interference  with  the  functions  of  the  cord  itself.  The  cord 
symptoms  consist  chiefly  in  impaired  conduction,  manifested  in  the 
j)arts  below  the  lesion.  The  central  functions  of  the  cord  (reflex 
a,ction,  &c.)  at  the  level  of  the  lesion  are  abolished  by  the  pressure,  but 
the  symptoms  of  this  abolition  are  often  lost  in  those  of  the  damage 
to  the  nerve-roots. 

Of  the  root  symptoms  the  most  constant  is  pain,  extending  along 
the  course  of  the  nerve-fibres,  and  through  the  area  of  their  distribu- 
tion. The  seat  of  these  pains  depends  on  the  position  of  the  disease ; 
they  may  be  felt  in  the  arms,  around  the  thorax  or  abdomen,  or  in 
the  legs.  They  are  usually  sharp  pains,  resembling  neuralgia  in 
character,  sometimes  accompanied  by  tender  points.  Occasionally, 
when  felt  in  the  limbs,  they  are  referred  especially  to  the  joints.  The 
pain  may  be  intermitting  or  constant.  It  may  even  be  absent,  as  in  a 
case  of  aneurism  of  the  arch  of  the  aorta  already  referred  to.  It  may 
exist  alone,  for  a  long  time,  in  a  disease  that  increases  slowly,  and 
sometimes  may  be  the  only  symptom,  although  some  compression  of 
the  cord  occurs.  With  it  there  is  usually  hyperaesthesia  of  the  skin, 
often  intense.  After  a  time  anaesthesia  develops  in  irregular  areas, 
in  spite  of  the  persistence  of  the  pain, — the  condition  termed 
"  anaesthesia  dolorosa."  Irritation  of  the  motor  nerve-roots  may  cause 
painful  contracture  of  the  muscles,  but  this  is,  on  the  whole,  rare,  and 
the  chief  motor  symptoms  are  due  to  the  interruption  of  the  fibres — 
muscular  weakness,  gradual  in  onset,  and  accompanied  by  wasting. 
The  rapidity  of  the  atrophy  varies  much,  and  with  it  the  electrical 
reaction.  When  slow,  there  may  be  merely  a  progressive  diminution 
in  irritability  to  f aradism  and  voltaism ;  when  rapid,  there  is  often  the 
degenerative  reaction,  and  sometimes  the  "  mixed  form,"  from  the 
partial  damage  to  the  fibres  that  supply  a  muscle. 

These  local  root  symptoms  are  usually  the  first,  and  to  them  are 
added,  after  a  time,  the  indications  of  interference  with  the  function 
of  the  cord  itself.  There  is  weakness  in  the  parts  supplied  from  the 
cord  below  the  lesion.  The  loss  of  power  is  usually  gradual,  bat 
sometimes  comes  von  rapidly,  even  in  the  course  of  a  few  hours,  when 
an  acute  myelitis  is  set  up  by  the  compression.  With  the  weakness 
there  is  a  marked  and  early  increase  in  the  superficial  reflex  action, 
more  constantly  conspicuous  than  in  most  other  diseases  of  the  cord, 
and  therefore  of  some  diagnostic  importance.  The  myotatic  irrita- 
bility is  also  increased,  a  foot-clonus  being  almost  always  present. 
Pains  in  the  legs  may  occur,  even  when  the  disease  is  above   the 


■391.  SPINAL   CORD. 

lumbar  enlargement,  usually  duU  and  aching.  There  is  often  formica- 
tion and  tingling.  There  may  be  no  impairment  of  sensibility  in  the 
parts  below  the  lesion,  even  when  there  is  complete  motor  palsy ;  in 
many  cases,  however,  there  is  some  sensory  loss,  complete  only  when 
the  spinal  lesion  is  very  severe.  Conduction  of  painful  impressions 
may  be  delayed,  sometimes  for  thirty  or  even  forty  seconds.  When 
the  pressure  is  lateral,  one  leg  may  be  first  and  most  affected,  but  the 
other  side  is  usually  soon  involved,  since  neither  the  mechanical  effect 
nor  the  inflammation  remain  limited  to  one  side  of  the  cord.  The 
sphincters  are  often  affected.  The  tendency  to  the  formation  of  bed- 
sores is  rarely  great,  unless  the  lumbar  enlargement  is  compressed  or 
myelitis  descends  into  it. 

The  course  of  the  symptoms  varies  according  to  that  of  their  cause. 
When  an  acute  myelitis  has  been  set  up,  improvement  may  occur  for 
a  time  even  if  the  cause  of  pressure  is  slowly  progressive.  This  is  true 
also  of  root  symptoms  when  they  are  rapidly  developed.  If  the 
compression  ceases  to  increase,  the  cord  may  recover  its  conducting 
function,  although  it  remains  narrowed.  Sensation  is  often  regained 
in  the  legs  when  motion  remains  absent,  but  both  motion  and  sensation 
may  be  regained  although  the  narrowing  of  the  cord  persists.  It  has 
even  been  found  no  larger  than  a  goose-quill  at  the  compressed  spot, 
although  the  paraplegia  had  passed  away.  In  such  a  case  many 
narrowed  nerve-fibres  are  found  in  the  section  of  the  compressed 
portion,  and  it  is  probable  that  many  axis-cylinders  persist,  with  an 
envelope  of  myelin  so  narrow  as  to  be  recognised  with  difficulty,  but 
which  sufiices  for  their  conducting  function.  Under  slow  pressure  the 
axis-cylinders  may  not  suffer  interruption,  although  the  myelin  dis- 
appears. Eegeneration  of  axis-cylinders,  as  in  the  peripheral  nerves, 
is  also  possible.  In  some  cases,  especially  when  a  considerable  myelitis 
has  been  set  up,  the  motor  path  may  recover,  but  the  posterior  colunmB 
remain  affected,  so  that  power  returns,  but  without  co-ordination,^ 
"  secondary  ataxy  "  resulting. 

The  Diagnosis  of  slow  compression  rests  on  the  root  and  cord 
symptoms  already  described,  and  especially  on  their  coincidence 
with  a  cause  of  compression.  Of  the  former,  the  sensory  symptoms 
are  the  most  characteristic.  In  many  cases  other  indications  of 
the  compressing  disease  assist  the  diagnosis.  These  vary  in  character  j 
the  most  frequent  is  considerable  local  tenderness  of  the  spine. 
If  root  symptoms  are  absent  the  diagnosis  is  much  more  difficult, 
unless  the  morbid  process  manifests  itself  externally.  If  there 
is  a  suspicion  of  compression  in  a  case  of  slow  paraplegia,  an  early  and 
considerable  excess  of  superficial  reflex  action  gives  additional  weight 
to  the  suspicion.  Even  without  any  other  symptoms  to  indicate  com- 
pression, this  is  suggested  by  symptoms  which  begin  on  one  side  and 
gradually  spread  to  the  other. 

The  chief  disease  from  which  compression  of  the  spinal  cord  has 
to  be  distinguished  is  a  subacute  or  subchronic  transverse  myelitis. 


ACUTE  ATEOPHIC  PAEALTSIS.  895 

Besides  tlie  indications  of  a  cause  of  compression  already  mentioned, 
the  chief  distinction  def^ends  on  the  presence  of  symptoms  indicatiug 
irritation  of  the  nerve-roots,  and  especially  on  the  fact  that  these  sym- 
toms  precede  those  of  the  lesion  of  the  spinal  cord  itself.  Although 
a  very  chronic  myelitis  is  occasionally  attended  by  much  irritation  of 
the  nerve-roots,  the  symptoms  of  this  succeed,  instead  of  preceding, 
the  damage  to  the  cord,  and  the  course  of  chronic  myelitis  is  often 
much  slower  than  is  that  of  most  forms  of  sluw  compression.  The 
distinction  from  a  growth  within  the  spinal  cord  rests  chiefly  on  the 
early  occurrence  of  impairment  of  the  central  and  conducting  func- 
tions of  the  cord,  and  also  on  the  partial  distribution,  and  often  slower 
course,  of  the  symptoms.  The  effects  of  an  intra-medullary  growth 
vary  widely  in  different  cases,  and  if  there  is  an  early  irritation  of  root- 
fibres,  the  distinction  may  have  to  depend  on  the  presence  or  absence 
of  the  signs  of  an  external  compressing  process. 

The  diagnosis  of  the  cause  of  compression  is  often  much  more 
difficult  than  that  of  its  occurrence  The  facts  that  the  patient  is  in 
the  first  half  of  life,  and  that  he  inherits  a  tubercular  tendency,  suggest 
caries.  Slightness  of  root  symptoms  is  also  in  favour  of  caries  or  a 
tumour  within  the  spinal  cord ;  the  presence  of  these  symptoms  does  not, 
however,  render  caries  less  likely,  unless  the  pain  is  extremely  severe 
and  is  greatly  increased  by  movement, — characters  that  should  always 
suggest  a  growth  in  the  bones  of  the  spine.  Eoot  symptoms  extending 
over  a  considerable  vertical  area  suggest  pachymeningitis.  Additional 
details  of  the  differential  diagnosis  will  be  found  in  the  account  of  the 
several  compressing  diseases. 

Peognosis  and  Teeatment. — The  prognosis  depends  on  the  cause, 
and  the  amenability  of  this  to  treatment.  As  a  general  mile,  however, 
the  more  rapidly  the  cord  symptoms  develop,  the  more  chance  there 
is  of  improvement,  because  the  greater  share  does  inflammation  take 
in  their  proc!uction.  The  treatm.ent  is  that  of  the  cause  of  the  com- 
pression, and  that  of  myelitis. 


ANTERIOR    POLIO-MYELITIS;    ATROPHIC    SPINAL 
PARALYSIS. 

In  certain  diseases  of  the  spinal  cord,  wasting  of  the  muscles  is  a 
prominent  symptom.  In  these  the  lesion  involves,  exclusively  or  in 
part,  the  anterior  grey  cornua,  and  the  muscular  atrophy  depends  on 
the  destruction  of  the  nerve-cells,  from  which  the  motor  nerve-fibres 
proceed,  and  on  the  consequent  degeneration  of  the  fibres.  The  mus- 
cular wasting  does  not  depend  on  the  nature  of  the  lesion ;  it  ocears 
equally  whether  the  nerve-cells  are  destroyed  by  haemorrhage,  by  in- 


896  SPIXAL    COHD. 

flammation,  acute  or  clirorLic,  by  slow  degeneration,  or  "by  presstire. 
But  tlie  rapidity  -witli  -wliich  tlie  destruction  occurs  influences  tlie 
character  of  the  symptom.  Time  is  required  for  the  change  in  the 
nutrition  of  the  muscles ;  one  or  two  weeks  elapse,  even  in  the  most 
acute  cases,  before  wasting  is  distinct.  The  nerve-cells  also  form 
part  of  the  voluntary  motor  path,  and  their  disease  at  once  interrupts 
this ;  hence,  in  acute  cases,  paralysis  is  rapidly  developed,  and  the 
atrophy  succeeds  it  after  an  interval.  In  chronic  lesions,  on  the  other 
hand,  the  changes  in  the  cells  and  muscles  progress  so  slowly  that  the 
weakness  and  wasting  appear  to  come  on  together.  The  wasting  may 
even  seem  to  precede  the  weakness,  but  a  careful  examination  will 
show  that  power  is  defective  as  soon  as  there  is  distinct  atrophy.  It 
is  customary  to  separate  the  two  groups  by  terming  those  in  which  the 
palsy  precedes  wasting,  "  atrophic  spinal  paralysis ;  "  and  those  in 
which  wasting  is  apparently  simultaneous  with  weakness,  "  spinal 
muscular  atrophy." 

The  atrophic  paralyses  are  due  to  an  acute  or  subacute  process  m 
the  anterior  cornua.  The  lesion  is  probably,  in  most  cases,  inflamma-- 
tory  in  character,  a  cornual  myelitis,  or  "  polio -myelitis  "  (Kussmaul). 
In  some  cases,  which  differ  chiefly  in  the  suddenness  of  the  onset,  it  is 
probable  that  the  lesion  is  haemorrhage  and  not  inflammation. 

The  chronic  atrophies  are  due  to  a  slow  degeneration  of  the  cells  and 
fibres.  Between  the  two  forms,  however,  we  meet  with  others  of  inter- 
mediate course,  subacute  or  subchronic,  due  to  processes  of  correspond- 
ing character, — ^usually  inflammation. 


Acute  Atrophic  PAHAiiTsis ;  Acxttb  Anteeioe  Polio-mtblitis. 

Infantile  Paralysis ;  Essential  Paralysis  of  Children. 

Acute  atrophic  paralysis  is  a  disease  in  which  voluntary  power  is 
lost  in  the  course  of  a  few  hours  or  days,  and  in  wliich  some  of  the 
paralysed  muscles  undergo  rapid  wasting  while  others  recover.  The 
onset  of  the  palsy  is  often  preceded  or  accompanied  by  indications  of 
general  disturbance.  The  muscles  which  waste  usually  remain  weak, 
and  contraction  of  their  opponents  may  lead  to  permanent  distortion 
of  the  paralysed  limbs.  Although  it  is  usually  a  form  of  acute 
myelitis,  its  characters  and  course  are  sufficiently  special  to  make  its 
separate  consideration  desirable.  The  disease  is  the  most  common 
form  of  paralysis  in  young  children,  and  has  hence  received  the  name 
of  "  infantile  paralysis."  Before  anything  was  known  of  the  lesion  on 
which  it  depends,  it  received  (from  EiUiet  and  Barthez)  the  unmean- 
ing designation  of  the  "  essential  paralysis  of  children,"  a  term  that  is 
falling  into  merited  disuse.  The  spinal  lesion  was  first  demonstrated 
by  Prevost  (1865),  and  soon  afterwards  by  Lockhart  Clarke  and  by 
Charcot  and  Joffroy.  Its  constancy  has  been  abundantly  proved  by 
numerous  observations  during  the  last  thirty  years.     The  occurrence  of 


ACUTE  ATEOPHIO  PARALYSIS.  397 

similar  symptoms  in  adults  was  noted  hj  Yogt  (1858)  and  Duclieime 
(1864). 

Etiology. — The  disease  occurs  at  all  ages,  but  is  ten  times  as 
frequent  in  the  first  decade  as  ia  all  the  rest  of  life.  It  is,  moreover, 
especially  a  disease  of  later  infancy.  Of  the  cases  in  early  life, 
three  fifths  occur  in  the  first  thi-ee  years  of  life,  nearly  one  fifth  in 
each  year.  The  numbers  in  each  successive  year  (of  214*  cases  of 
which  I  have  notes)  were — ia  the  first  54,  the  second  58,  third  39, 
fourth  17,  fifth  23,  sixth  8,  seventh  4,  eighth  6,  ninth  5.  Of  the 
cases  that  begin  during  the  first  year,  nearly  all  begin  in  the  second 
six  months ;  the  earliest  of  the  series  occurred  at  two  months,  but 
one  at  the  twelfth  day  is  described  by  Duchenne.  The  friends  often 
date  the  disease  from  birth,  but  there  is  no  real  evidence  of  its  intra- 
uterine development.  After  childhood  it  is  most  frequent  between 
ten  and  twenty,  and  very  rare  over  forty.  One  case,  however, 
occurred  at  sixty-three.  Many  supposed  subacute  cases  in  adults 
have  been  examples  of  multiple  neuiitis. 

In  childhood  the  two  sexes  suffer  nearly  equally  (males  133,  females 
103,  in  236  cases),  but  after  ten  the  malady  is  almost  confined  to  the 
male  sex.  The  influence  of  heredity  is  small,  but  a  family  tendency  to 
suffer  may  now  and  then  be  traced ;  two  and  even  three  brothers  or 
sisters  have  been  affected  (Seeligmiiller,  &c.).  One  of  my  own  patients 
had  a  sister,  and  another  two  cousins,  affected  with  the  same  disease. 
When  more  members  of  a  family  than  one  are  affected,  they  usually 
suffer  simultaneously.  The  inherited  tendency  may  be  indirect;  of 
a  patient  in  whom  the  disease  came  on  in  adult  life,  two  brothers  had 
suffered  from  hemiplegia  and  an  uncle  from  paraplegia. 

The  disease  presents  a  remarkable  relation  to  season,  occurring  far 
more  frequently  ia  summer  than  in  winter.  Sinkler,  who  first  called 
attention  to  the  fact,  found  that  in  Philadelphia  four  fifths  of  the 
cases  commenced  in  the  five  hot  months.  May  to  September.  In  this 
country  the  relation  is  equally  apparent ;  three  quarters  of  the  cases 
occur  during  the  hottest  third  of  the  year,  June  to  September.  The 
distribution  of  160  cases  was  as  follows: — 3  occurred  in  January, 
5  in  February,  in  April  2,  May  3,  June  22,  July  32,  August  37, 
September  25,  October  15,  November  8,  December  8.  In  many  cases 
the  weather  was  very  hot  at  the  time ;  in  some,  however,  the  disease 
came  on  in  cooler  weather  after  a  hot  period.  It  would  therefore 
seem  that  the  heat  generally  acts  as  a  remote  cause,  and  is  only 
occasionally  concerned  directly  in  the  exciting  mechanism.  The  fact  is 
the  more  important  because  cold  has  long  been  regarded  as  the  most 
potent  exciting  cause.  That  exposure  to  cold  is  an  occasional  cause  is 
certain,  although  the  number  of  cases  in  which  it  can  be  traced  is  not 
large ;  and  it  is  usually  a  general  and  not  a  local  exposure,  and  has  not 
often  been  immediately  preceded  by  exposure  to  heat.     Exceptions  are 

*  of  these  214  cases  116  were  seen  and  analysed  by  Sir  W.  Gowers,  and  98  by 
Dr.  James  Taylor.     I'hey  include  both  hospital  and  private  cases. 


398  SPINAL    COED. 

chiefly  met  with  in  adults.  Thus  one  patient,  aged  sixteen,  when 
perspiring  from  a  long  ride  after  the  hounds  on  a  hot  day  in  September, 
lay  down  on  a  sofa  beneath  an  open  window  from  which  a  draught 
blew  on  his  back.  Two  days  afterwards  the  first  symptoms  com- 
menced. In  another  case  the  onset  followed  a  day  on  a  steamer  in 
cold,  wet  weather,  preceded  by  much  paddling  in  the  sea  during 
extreme  heat.  I  have  several  times  known  the  disease  to  be  due  to 
sitting  on  damp  grass.  Two  or  three  days  is  the  interval  which  usually 
elapses  between  the  exposm-e  to  cold  and  the  onset  of  the  symptoms, 
but  great  variations  are  met  with,  and  the  constitutional  disturbance 
may  commence  within  twenty-four  hours  of  the  exposure. 

Over-exertion  in  walking  seems  occasionally  to  aid  in  exciting  the 
disease ;  in  some  cases  the  child  has  walked  far  more  than  was  proper 
the  day  before  the  onset,  and  in  others  habitual  over-exertion  has  been 
permitted.  A  traumatic  influence,  such  as  a  fall,  occasionally  pre- 
cedes the  onset  in  such  a  way  as  to  make  its  influence  probable. 
Thus,  in  one  severe  case,  a  boy  eight  years  old  was  thrown  over  the 
head  of  a  donkey,  and  the  onset  occurred  five  days  afterwards.  A 
girl  of  fonr  fell  off  a  chair,  striking  her  head,  and  was  stunned  for  a 
moment ;  two  days  later  she  was  sipk  and  feverish ;  the  temperature 
(101°)  continued  raised  till  the  sixth  day,  when  general  loss  of  power 
was.  found,  and  the  residual  state  was  atrophic  palsy  of  both  legs  and 
the  right  deltoid.  The  cases  following  falls  and  over-exertion  present 
the  same  relation  to  season  as  do  the  rest. 

There  is  no  proof  of  any  relation  between  the  disease  and  the 
process  of  dentition.  The  period  of  dentition  coincides  with  the  rapid 
functional  development  of  the  nervous  system  which  succeeds  its 
structural  development  in  the  first  few  months  of  life.  Moreover 
this  period  is  often  one  of  deterioration  of  health,  in  consequence  of 
changes  of  diet  and  other  causes.  The  disease  occurs  before  and 
after  the  period  of  teething,  and  it  exhibits  no  increase  in  frequency 
at  the  period  of  the  second  dentition. 

Many  children  are  perfectly  well  at  the  time  when  the  disease 
occurs.  A  few  are  in  conspicuously  defective  health.  Thus  in  one 
case  the  child  had  been  rendered  feeble  by  long-continued  diarrhcea. 
I  have  only  once  seen  the  disease  in  the  subject  of  inherited  syphUis. 
It  has  been  thought  to  be  occasionally  secondary  to  acute  febrile 
diseases,  but  the  frequency  of  the  relation  has  been  unquestionably 
overrated ;  in  some  supposed  cases  in  adults  there  has  been  multiple 
neuritis  only,  and  in  children  initial  general  disturbance  is  constantly 
mistaken  for  an  independent  general  disease,  and  the  opinion  is  often 
maintained  after  the  discovery  of  the  paralysis,  which  is  then  sup- 
posed to  be  of  secondary  origin.  Thus  in  one  case  initial  pyrexia, 
headache,  and  vomiting  were  supposed  to  indicate  scarlet  fever. 
Paralysis  of  all  four  limbs  came  on,  with  difficulty  in  swallowing,  and 
the  latter  was  supposed  to  render  certain  the  diagnosis  of  scarlet 
fever,  although  there  was  no  sore  throat  or  rash.     In  older  children 


ACUTE    ATliOPHIO    PAEALTSIS.  b99 

and  adults  tlie  disease  is  often  thouglit  to  be  secondary  to  rheumatic 
fever,  in  consequence  of  the  rheumatoid  character  of  the  pains.  The 
error  is  the  more  likely  to  occur  if  the  symptoms  follow  exposure  to 
cold.  It  is  conceivable  that  catarrhal  affections  may  be  produced  by 
the  exposure  to  cold  which  induces  the  disease ;  but  of  the  214  cases 
referred  to  above,  not  one  was  related  to  any  acute  specific  disease, 
or  to  rheumatic  fever.  Chronic  alcoholism  has  been  thought  to  be 
a  cause  in  adults,  but  it  is  probable  that  most  cases  supposed  to  be 
due  to  this  influence  have  been  instances  of  multiple  neuritis. 

Symptoms. — The  general  characters  of  the  symptoms  have  been 
already  mentioned — an  acute  onset,  often  with  constitutional  disturb- 
ance ;  paralysis,  at  first  wide-spread,  afterwards  passing  away,  except 
from  a  region  in  which  the  muscles  rapidly  atrophy,  and  in  which, 
although  partial  recovery  may  occur,  more  or  less  weakness  and  wasting 
persist. 

The  general  disturbance,  which  is  apparently  due  to  a  morbid 
blood-state,  may  be  severe  or  slight.  In  most  cases  there  is  pyrexia, 
with  its  usual  accompaniments,  headache,  prostration,  loss  of  appe- 
tite, restlessness.  Vomiting  is  very  common,  and  often  causes  the 
illness  to  be  mistaken  for  stomach  disturbance.  Occasionally  there 
is  also  diarrhoea.  These  symptoms  of  general  disturbance  usually 
last  for  a  few  days,  sometimes  for  only  a  few  hours,  and  they  are 
occasionally,  but  not  often,  absent.  The  palsy  develops  in  most  cases 
in  the  course  of  this  disturbance.  A  common  history  is  for  a  child 
to  seem  ill  and  feverish,  to  be  put  to  bed,  and  the  next  morning  to 
be  found  to  have  lost  power.  In  other  cases  the  febrile  disturbance 
lasts  for  several  days  before  the  paralysis  comes  on.  But  the  relation 
of  the  general  disturbance  to  the  onset  of  the  paralysis  presents 
great  variations,  which  appear  (from  a  comparison  of  cases)  to  be 
independent  of  any  variations  in,  or  special  features  of,  the  spinal 
symptoms.  The  constitutional  symptoms  occasionally  succeed  the 
onset.  Thus  a  child,  well  one  day,  was  found  next  morning  to  have 
both  legs  paralysed,  and  was  feverish  and  sick ;  the  general  disturb- 
ance continued  for  a  week.  The  elevation  of  temperature  is  usually 
moderate  in  degree ;  an  example  of  its  character,  in  what  may  be 
legarded  as  a  fairly  typical  case,  is  that  of  a  child  whose  tempera- 
ture on  the  second  day  of  illness  was  101"2°  ;  on  the  third,  101°;  the 
fourth  (on  which  the  palsy  developed),  100'';  the  fifth,  99-5°;  the 
sixth,  98°.  It  is  usually,  as  in  this  case,  highest  at  the  commencement, 
and  sometimes  reaches  103°,  104°,  or  even  in  rare  cases  105°.  The 
constitutional  symptoms  are  present  in  most  of  the  cases  in  which  a 
fall  appears  to  excite  the  disease,  and  are  of  the  usual  character ;  they 
are  as  marked  in  the  cases  that  appear  to  be  spontaneous  as  in  those 
that  follow  exposure  to  cold. 

Convulsions  occasionally  attend  the  onset,  chiefly  in  young  children. 
In  soniP  instances,  moreover,  the  initial  symptoms  are  those  of  sevei"© 
cerebral  disturbance.     Thus  one  child  had  repeated  general  convul- 


400  SPINAL    COED. 

sions  during  thirty  hours,  and  afterwards  was  in  a  state  of  deep  coma 
for  thirty-six  hours,  the  temperature  at  the  onset  "being  105°.  This 
was  succeeded  by  slighter  pyrexia  and  prostration  for  three  weeks, 
and  it  was  only  at  the  end  of  that  time  that  general  loss  of  power  was 
found  to  have  come  on.  The  arms  recovered,  but  atrophic  palsy  per- 
sisted in  the  legs.  In  another  case,  a  child  of  seven  months  became 
ill,  febrile  (102-8°)  and  comatose  with  pin-point  pupils;  it  remained 
ia  that  state  for  four  or  five  days,  and  then  gradually  recovered,  but 
with  general  powerlessness,  rigidity  of  the  legs,  and  tenderness  of 
legs  and  back.  The  rigidity  and  pain  passed  away,  but  the  right 
deltoid  and,  to  a  less  degree,  the  biceps  and  triceps,  and  the  whole  of 
the  right  leg  remained  paralysed,  and  presented  characteristic 
atrophy.  In  adults  there  is  sometimes  marked  somnolence  before  the 
onset,  and  occasionally  slight  cerebral  symptoms,  such  as  diplopia, 
giddiness,  or  (very  seldom)  delirium.  A  medical  student  found,  one 
day,  that  he  saw  double ;  on  the  next  day  he  felt  giddy,  and  became 
feverish  (102°) ;  he  slept  for  forty-eight  hours,  and  on  the  fourth  day 
found  his  right  arm  was  weak ;  atrophy  of  the  deltoid,  &c.,  followed. 
The  cerebral  symptoms  usually  pass  away  together  with  the  indica- 
tions of  general  disturbance,  and  hence  are  probably  due  to  the  blood- 
statue,  but  occasionally  slight  facial  weakness  has  persisted  for  several 
weeks.  In  rare  cases  the  onset  is  accompanied  by  symptoms  of 
slight  transient  spinal  meningitis,  which  must  be  regarded  as  a 
coincident  effect  of  the  cause,  since  the  membranes  may  be  affected 
early.  Thus  a  child,  after  being  feverish  and  vomiting  for  two  days, 
became  universally  rigid,  so  that  strychnine  poisoning  was  thought  of. 
The  rigidity  passed  away  two  days  later,  when  the  right  arm  and  leg 
were  found  to  be  paralysed.  Another  child  presented  all  the  sym- 
ptoms of  spinal  meningitis  a  week  after  the  onset  of  paralysis  of  the 
arm,  and  as  they  passed  off  the  leg  also  was  found  affected.  When 
the  general  illness  is  severe,  the  paralysis  is  often  not  discovered  for 
some  time,  especially  in  a  child ;  the  immobility  of  paralysis  is  often 
mistaken  for  the  inertia  of  prostration. 

In  older  children  and  adults  another  common  initial  symptom  is 
the  pain  already  mentioned,  sometimes  referred  to  the  muscles,  some- 
times to  the  limbs  generally,  occasionally  to  the  course  of  the  nerves. 
It  is  often  absent,  but  may  be  very  severe  and  accompanied  by  extreme 
tenderness  of  the  limbs.  In  such  cases  the  pain  may  be  supposed  to 
be  in  the  joints,  because  it  is  produced  by  moving  them,  when  it  is 
really  in  the  nerves,  which  are  mechanically  disturbed  by  the  move- 
ment of  the  joints.  In  these  cases  there  is  neuritis,  which  may  come 
on  at  the  same  time  as  the  palsy,  although  independent  of  it.  The 
joints  themselves  may  be  the  seat  of  pain,  and,  when  the  exposure  to 
cold  has  been  severe,  inflammation  of  one  or  more  joints  has  been 
known  to  come  on  soon  after  the  onset.  Pain  may  be  felt  also  in  the 
back,  usually  dull  and  achiag.  It  may  be  severe,  especially  when  thei-e 
are  symptoms  of  spinal  meningitis.    Instead  cf  being  initial,  pain  in 


ACUTE  ATROPHIC  PARALYSIS.  401 

the  limits  may  succeed  the  onset  of  paralysis,  and  it  occasionally  con- 
tinues for  several  weeks,  especially  when  seated  in  the  nerve-trunks. 
In  some  cases  in  which  the  onset  and  course  are  characterised  by 
severe  pains,  the  motor  effects  have  entirely  disappeared  in  course  of 
time,  indicating  that  the  condition  was  one  of  neuritis.  Tingling  or 
formication  in  the  limbs  may  be  described  by  older  patients,  but  sen- 
sation is  seldom  affected,  even  temporarily. 

The  paralysis  is  almost  always  rapidly  developed.  It  varies  much 
in  its  initial  range.  Only  part  of  a  limb  may  be  affected,  or  there 
may  be  universal  loss  of  power ;  but  in  the  majority  of  cases  the  para- 
lysis is  intermediate  between  these  extremes.  Two  or  three  limbs  are 
affected — both  arms,  both  legs,  or  the  legs  and  one  arm.  When  aU 
four  limbs  are  paralysed  the  neck  muscles  also  may  be  weak,  and  even 
swallowing  may  be  impaired.  The  other  muscles  supplied  by  the 
cranial  nerves  escape,  as  a  rule,  and  the  sphincters  are  seldom  involved. 
Occasionally  there  is  retention  of  urine,  sometimes  incontinence,  chiefly 
ia  severe  cases,  and  it  may  then  last  a  long  time.  For  instance,  a  child 
aged  two  and  a  half  years  woke  up  one  morning  with  headache,  fever, 
and  weakness  of  the  legs,  which  rapidly  increased  to  complete  paralysis. 
Four  days  later  the  arms  also  became  weak,  and  in  a  day  or  two  more 
the  urine  escaped  involuntarily.  The  arms  began  to  recover  in  six 
weeks,  and  were  well  in  six  months :  both  legs  wasted  and  remained 
permanently  paralysed ;  the  incontinence  of  urine  lasted  for  a  year. 
In  another  case,  with  incontinence  for  a  few  weeks,  the  residual  palsy 
was  in  the  lower  legs.  As  in  these  instances,  the  symptom  is  met 
with  chiefly  when  the  lumbar  region  is  diseased. 

The  paralysis  may  commence  in  one  limb  and  quickly  spread, 
reaching  its  maximum  extent  in  a  few  hours  to  a  few  days,  but  very 
often  the  actual  onset  is  so  rapid  or  so  escapes  careful  observation  in 
consequence  of  the  general  illness  that  all  the  parts  first  affected  are 
found  to  be  paralysed  when  it  is  discovered.  Occasionally  the  onset 
is  in  two  distinct  stages,  separated  by  a  few  days ;  sometimes  the  loss 
of  power  develops  slowly  in  the  course  of  one  or  even  two  weeks,  in 
cases  that  must  be  regarded  as  "  subacute,"  but  do  not  differ  in  other 
respects  from  the  ordinary  form.  Examples  of  the  common  onset  have 
been  already  given ;  as  another,. may  be  mentioned  the  case  of  a  boy, 
aged  a  year  and  four  months,  who  one  day  seemed  ill,  was  sick,  and 
was  put  to  bed.  The  next  day  he  could  scarcely  stand  alone ;  on  the 
following  day  he  could  neither  move  the  legs  nor  sit  up  in  bed.  In 
about  ten  days  the  left  leg  began  to  improve,  but  there  was  enduring 
paralysis  and  wasting  of  the  whole  of  the  right,  and  of  the  lower  part 
of  the  left  leg.  In  another  case,  a  child  seemed  Ul,  and  was  kept  in 
bed  for  five  days ;  on  the  third  day  it  was  noticed  that  the  left  arm 
was  not  moved  so  much  as  the  right,  and  by  the  fifth  day  the  arm  was 
quite  powerless ;  at  the  end  of  a  fortnight  improvement  commenced. 
In  the  adxdt,  the  mode  of  onset  presents  nearly  the  same  characters. 
A  lady,  twenty -five  years  of  age,  sat  down  for  some  time  on  wet  grass^ 
VOL.  1.  26 


402  SPINAL   COUD. 

Two  days  later  general  rheumatic  pains  came  on,  "wlaicli  were  very  severe 
in  the  legs  on  the  following  day,  and  the  legs  were  distinctly  weak.  In 
the  course  of  the  next  forty -eight  hours  the  arms  also  became  feeble,  and 
at  the  end  of  a  week  she  could  scarcely  move  her  arms,  and  her  legs 
were  absolutely  paralysed.  In  a  fortnight  improvement  commenced, 
first  in  the  arms  and  then  in  the  feet ;  but  the  hip  and  thigh  muscles 
remained  paralysed  and  rapidly  wasted. 

Cases  are  sometimes  seen  similar  in  other  respects  to  those  now 
under  consideration,  but  in  which  the  paralysis  comes  on  suddenly, 
and  without  general  disturbance.  Thus  a  child,  aged  two,  was  walking 
along,  when  he  suddenly  could  not  stand,  and  fell  down  on  his  knees ; 
both  legs  were  powerless.  Slow  improvement  followed  in  the  left  leg, 
and  wasting  in  the  right.  The  suddenness  of  the  onset  makes  it 
probable  that  the  lesion  is  haemorrhage,  and  not  simple  inflammation. 
Spinal  haemorrhage  is  known  to  occur  in  early  childhood.  Hence, 
while  we  must  class  these  with  the  other  acute  spinal  atrophies,  we  are 
not  justified  in  regarding  them  as  cases  of  polio-myelitis,  since  we  do 
not  know  whether  the  haemorrhage  is  secondary  to  commencing  inflam- 
mation or  is  primary.  The  former  is  probable,  because  the  two  sets  of 
cases  occur  under  the  same  general  conditions  of  onset,  and  hence  they 
cannot  be  separated.  Moreover,  constitutional  disturbance  is  more 
often  absent  in  cases  of  actually  sudden  onset  than  in  others,  although 
it  is  sometimes  considerable.  Thus  a  child  had  been  ill  for  two  weeks, 
when,  being  able  to  walk  about,  it  suddenly  fell  to  the  ground ;  the 
left  leg  was  found  powerless,  and  the  right  weak.  It  is  probable  that 
a  sudden  onset  is  more  frequent  than  is  suggested  by  the  facts  that 
can  be  ascertained,  since  the  palsy  often  comes  on  during  the  night  or 
during  rest ;  for  instance,  in  one  case  a  child  sat  on  a  chair  for  an 
hour,  when  the  left  leg  was  found  powerless. 

In  some  cases  of  sudden  onset  the  initial  palsy  is  universal,  and  in 
such  cases  as  the  following  it  is  probable  that  a  limited  haemorrhage 
in  the  cervical  region  causes  rapid  pressure  on  all  the  adjacent 
structures,  which  afterwards  recover  from  the  effects  of  the  pressure. 
A  girl,  aged  seventeen,  suddenly  felt  tingling  in  the  left  hand.  In 
a  few  minutes  the  whole  arm  was  powerless,  and  then  the  right  arm 
became  paralysed.  She  had  a  strange  sensation  at  the  back  of  the 
neck,  and  went  upstairs ;  as  she  was  going  up,  her  legs  became  weak, 
first  the  left  and  then  the  right.  In  less  than  half  an  hour  from  the 
time  of  the  first  symptom,  there  was  absolute  universal  paralysis,  with 
difficulty  in  breathing  and  in  swallowing.  Improvement  (in  the 
opposite  order  to  the  onset)  commenced  the  same  evening  and  was 
complete  in  about  six  weeks,  with  the  exception  of  the  muscles  of  the 
left  forearm  and  hand,  which  rapidly  wasted  and  were  permanently 
paralysed  and  atrophied.  Another  girl,  while  walking  across  a  road, 
suddenly  felt  a  "  sort  of  shock  "  as  if  someone  had  given  her  a  knock 
between  the  shoulders.  She  became  giddy,  and  instantly  felt  tingling 
in  both  arms,  especially  in  the  right,  which  became  weak  before  she 


ACUTE  ATROPHIC  PARALYSIS.  403 

had  got  to  the  other  side  of  the  road.  "Ultimately  the  arms  recovered, 
but  permanent  paralysis  and  wasting  of  the  intrinsic  muscles  of  each 
hand  were  left. 

The  initial  loss  of  power  is  as  a  rule  far  more  extensive  and  severe 
than  the  permanent  affection;  iu  a  small  number  of  cases  the 
two  correspond.  But  the  extent  of  the  early  palsy,  that  of  the 
enduring  atrophy,  and  the  relation  between  the  two,  vary  extremely, 
so  that  no  general  or  even  common  laws  can  be  discovered.  Indeed, 
in  all  features  of  the  disease  variability  prevails  to  such  an  extent 
as  to  baffle  attempts  to  describe  them  in  general  terms,  and  a  better 
conception  of  the  malady  can  be  gained  from  such  illustrative  instances 
as  are  here  given  than  from  general  descriptions.  One  general  fact 
is  unfortunately  true  of  almost  all  cases,  namely,  that  the  palsy 
scarcely  ever  passes  away  entirely,  and  that  where  wasting  occurs, 
wasting  remains. 

After  the  paralysis  has  reached  its  height  and  ceased  to  increase  or 
spread,  whether  the  poiut  is  attained  suddenly  or  rapidly  or  slowly, 
it  remains  stationary  for  a  time,  which  varies  from  a  few  days  to  six 
weeks,  and  then  lessens.  The  improvement  occurs  first  in  the  parts 
last  affected,  and  gradually  spreads  until,  usually  at  the  end  of 
Erom  one  to  three  months,  all  parts  have  recovered  except  those  which 
are  to  be  permanently  affected.  In  these  the  muscles  are  toneless  and 
flaccid  from  the  first,  and  in  two  or  three  weeks  there  is  distinct  wast- 
ing, which  rapidly  increases,  until  the  shape  of  the  limb  is  changed, 
and,  in  extreme  cases,  scarcely  any  of  the  volume  of  the  muscle  can 
ultimately  be  detected.  In  fat  children  the  appearance  of  the  limb 
may  be  less  altered,  and  it  seems  that,  in  some  of  these,  an  interstitial 
growth  of  fat  makes  up  for  the  diminution  of  muscular  tissue.  In 
older  children  and  adults  the  muscles  are  often  tender  to  the  touch 
during  the  process  of  wasting. 

When  the  atrophy  is  distinct  the  muscles  no  longer  contract  to 
faradism,  and,  if  the  motor  nerves  are  tested,  they  also  will  be  found 
to  have  lost  irritability.  The  change  depends  on  degeneration  of 
the  nerves,  and  the  muscles  usually  present  the  "degenerative 
reaction  "  in  its  characteristic  form.  The  loss  of  f aradic  irritability  is 
distinct,  in  severe  cases,  as  early  as  the  end  of  the  first  week,  and  even 
by  the  fifth  or  sixth  day.  In  a  patient,  for  instance,  who  is  Tiniversally, 
and  apparently  uniformly,  paralysed,  one  or  more  groups  of  muscles 
may  be  found  to  have  lost  irritability,  and  in  these  we  know  that  there 
wiU.  be  lasting  paralysis  and  wasting,  while  the  other  parts  will  recover. 
In  a  severe  case,  in  which  the  muscles  most  affected  atrophy  completely, 
the  loss  of  faradic  irritability  may  be  permanent.  The  voltaic  iiTita- 
biUty  remains,  increased  in  degree,  for  two,  three,  four,  or  six  months, 
then  slowly  falls,  as  the  muscular  fibres  themselves  degenerate,  and 
ultimately,  at  the  end  of  one  or  two  years,  it  disappears.  It  may, 
however,  persist  for  a  long  time,  if  the  fibres  are  stimulated  by  elec- 
tricity,— even  for  three  or  four  years,  although  the  paralysis  remains 


404  SPINAL    COKD. 

absolute.  In  rare  cases,  electrical  irritability  quickly  disappears,  even 
to  the  voltaic  current,  from  the  destructive  degeneration  of  the 
muscular  fibres.  More  commonly,  after  six  or  twelve  months,  some 
faradic  irritability  returns.  It  may  be  slight,  due  to  the  recovery  of 
a  few  fibres,  insufficient  in  number  to  restore  any  bulk  or  power,  but 
the  new  fibies  which  recover  do  so  perfectly,  so  that  the  irritability 
becomes  normal  in  degree,  although  the  contraction  which  can  be  thus 
produced  is  very  slight  in  amount.  In  other  cases  considerable 
recovery  occurs,  so  that  some  power  and  volume  are  regained, 
although  the  muscles  remain  below  the  normal  size.  In  the  muscles 
which  are  weak  but  do  not  waste  there  is  no  degenerative  reaction, 
although  there  may  be  slight  diminution  of  excitability  to  each 
current. 

The  paralysis,  as  a  rule,  is  motor  only.  Sensation  is  impaired  only 
in  extremely  rare  cases  (about  1  in  50),  in  which  inflammation  in  the 
lumbar  region  is  so  intense  as  temporarily  to  impair  all  the  conduct- 
ing functions-  of  the  cord.  Thus,  in  a  girl  aged  five,  in  whom  the 
permanent  state  was  a  symmetrical  atrophy  of  all  the  muscles 
below  the  knees,  with  a  slighter  affection  of  the  thigh-muscles  and 
flexors  of  the  hips,  the  initial  general  loss  of  power  in  the  legs  was 
accompanied,  for  a  few  days,  by  complete  loss  of  sensation  below  the 
knees.  In  the  similar  but  more  severe  case  of  a  boy,  aged  four,  in 
whom  the  lasting  palsy  was  general  throughout  both  legs,  the  anaes- 
thesia continued  for  two  months.  In  many  cases  there  is  a  history  of 
temporarily  disturbed  sensation  attending  the  onsd:,  probably  the 
result  of  oedema  around  the  inflammatory  area.  When  sensation  is 
lost  there  is  always  incontinence  of  urine. 

Eeflex  action  necessarily  ceases  in  the  parts  related  to  the  affected 
muscles.  That  from  the  skin  is  at  first  lost  where  there  is  weakness, 
but  it  returns  with  or  soon  after  the  recovery  of  power  in  the  less 
affected  parts.  Where  there  is  persistent  paralysis  it  remains  absent. 
The  myotatic  irritability  is  lost  in  the  same  or  even  greater  degree. 
For  instance,  no  knee-jerk  can  be  obtained  if  the  extensors  of  the 
knee  are  affected  even  in  slight  degree.  The  loss  depends  on  the 
interruption  of  the  muscle-reflex  centre  by  the  disease  in  the  grey 
matter.  In  rare  cases  of  severe  cervical  polio-myelitis,  the  inflam- 
mation spreads  beyond  the  grey  matter  to  the  lateral  white  columns ; 
there  is  wasting  of  the  arms,  but  paralysis  without  wasting  in  the 
legs,  and  in  the  latter  the  myotatic  irritability  may  be  increased 
above  the  normal,  so  that  the  foot-clonus  may  be  obtained.  In  the 
course  of  a  few  months,  however,  the  condition  of  the  legs  becomes 
normal. 

A  common  effect  of  the  disease  is  to  retard  the  growth  of  the  bones 
in  the  affected  limb,  so  that  these  gradually  become  shorter  than  their 
fellows,  and  the  difference  in  size  increases  with  the  amount  of  general 
growth.  Hence,  it  is  greater,  the  younger  the  patient  at  the  onset  of 
the  disease.    If  one  part  of  a  limb  is  more  affected  than  another,  the 


ACUTE  ATEOPHIC  PARALYSIS.  405 

corresponding  bone  suffers  most  in  growth.  Tlie  effect  is  sometimes 
considerable,  when  the  atrophy  is  slight.  Seeligmiiller  has  described 
the  opposite  effect — an  actual  elongation  of  the  bones,  attributed 
to  the  fact  that  the  growing  epiphyses  suffer  traction  instead  of  the 
normal  compression,  but  this  is  extremely  rare. 

Those  joints  that  depend  for  their  support  on  tendons  which  pass 
over  them  become  lax  when  the  muscles  are  paralysed,  and  the 
articular  surfaces  may  be  no  longer  kept  together.  Thus  when  the 
deltoid  is  wasted  the  head  of  the  humerus  may  fall  out  of  the  glenoid 
cavity.  The  head  of  the  femur  may  fall  from  its  proper  position  in 
the  acetabxilum,  and  actual  luxation  may  occur  when  the  paralysis  of 
the  muscles  is  unequal,  and  the  less  affected  unopposed  muscles 
undergo  the  secondary  contracture  immediately  to  be  described. 
That  of  the  abductors  and  rotators  may  cause  infra-pubic  dislocation, 
and  that  of  the  adductors  iliac  dislocation.*  I  have  known  the  patellar 
tendon  to  be  greatly  elongated,  from  the  unopposed  action  of  the 
extensors  of  the  knee,  so  that  their  contraction  caused  the  patella  to 
rise  three  inches  above  its  normal  position. f 

During  the  chronic  stage  of  the  disease  there  is  great  tendency  to 
the  occurrence  of  permanent  shortening  of  muscles,  with  consequent 
displacement  of  the  parts  to  which  they  are  attached.  Grave  de- 
formities are  thus  produced.  It  occurs  only  when  the  several  muscles 
acting  on  a  joint  are  unequally  paralysed ;  the  less  affected  muscles 
are  no  longer  subject  to  the  normal  extension  by  their  opponents,  and 
hence  gradually  become  contracted,  and  the  contraction  is  fixed  by 
tissue  changes  in  them,  so  that  they  cannot  be  passively  extended. 
It  may  occur  in  muscles  which  are  unaffected,  and  it  is  uncertain  how 
far  it  is  favoured  by  a  slight  degree  of  paralysis.  Posture  also  aids 
the  production  of  those  muscular  contractions,  and  so  also  in  the  case 
of  the  foot  does  the  relative  shortening  of  the  limb. 

Bedsores  are  almost  unknown,  even  in  the  acute  stage  of  the  disease. 
A  slight  local  elevation  of  temperature  in  the  most  affected  parts  has 
been  observed  in  the  early  stage,  but  subsequently  the  affected  limb 
is  colder  than  the  other.  This  is  due,  in  part  at  least,  to  the  want 
of  the  aid  to  the  circulation  which  is,  in  health,  supplied  by  the 
muscular  action. 

The  persistent  paralysis  may  occupy  the  whole  or  part  of  one  or 
more  limbs.  The  legs  suffer  three  times  as  frequently  as  the  arms ; 
the  respective  percentage  being  21  and  68.  It  is  less  frequent  for  both 
legs  or  for  one  arm  to  suffer,  and  still  rarer  combinations  are  those  of 
both  legs  and  one  arm,  of  both  arms,  of  the  arm  on  one  side  and  the 
leg  on  the  other,  while  the  rarest  of  all  is  the  affection  of  leg  and  arm 
on  the  same  side.  The  limbs  on  the  left  side  of  the  body  are  more 
often  permanently  affected  than  those  on  the  right ;  the  difference  is 

•  See  Karewski,  *  Archiv  f.  klin.  Chir./  Bd.  xxxvii. 
t  In  a  case  shown  me  by  Mr.  Heather  Bigg. 


406 


SPINAL    CORD. 


greater  in  tLe  leg  than  in  the  arm.     The  left  leg  is  paralysed  alone 
twice  as  often  as  the  right. 

When  the  paralysis  of  a  limb  is  incomplete,  the  part  involved  varies 
much  in  different  cases,  and  as  different  parts  of  two  or  more  limbs 
may  be  affected,  the  combinations  of  palsy  which  result  are  extremely 
varied.  The  grouping  of  adjacent  muscles  is  sometimes  distinctly  that 
of  functional  association ;  often  it  is  random ;  but  the  most  frequent 
condition  is  to  have  an  irregular  affection  of  the  muscles  that  are 
associated  in  the  centre.  While  the  grouping  is  that  of  function, 
some  of  the  muscles  are  only  slightly  affected,  and  others  suffer  with 
them  that  are  not  functionally  associated.  In  the  legs,  the  paralysis, 
in  very  severe  and  fortunately  very  rare  cases,  may  be  absolute, 
involving  all  the  muscles  of  both  hmbs.  Usually  it  is  partial,  and 
then  the  muscles  below  the  knee  suffer  more  often  than  those  above 

the  knee.  The  calf  muscles 
are  affected  less  frequently 
or  in  less  degree  than  the 
muscles  in  front  of  the  tibia 
or  the  peronei.  Hence  talipes 
equinus  is  a  common  defor- 
mity,  and  its   occurrence  is 


'^^M, 


i 


Fig.  112. —  Old  atrophic  spinal  paralysis. 
Talipes  valgus  iii  the  right  leg  from  para- 
lysis of  the  anterior  tibial ;  t.  varus  in  the 
left,  from  paralysis  of  the  peronei. 


^ 


Fia.  113. — Atrophic  spinal  para- 
lysis, affecting  cliiefly  the  calf 
muscles,  with  resulting  contrac- 
tion of  the  flexors  of  the  foot, 
causing  talipes  calcaneus. 


aided  by  the  shortening  of  the  limb,  in  consequence  of  which  the 
foot  has  to  be  extended  to  bring  the  ball  to  the  ground.  Either 
the  tibialis  anticus  or  the  peronei  may  be  more  affected,  with  the 
result,  in  the  former  case,  of  tahpes  valgus,  in  the  latter  of  talipes 
varus.  In  the  case  shown  in  Fig.  112  the  peronei  have  suffered  most 
in  the  left  leg  and  least  in  the  right,  producing  an  unsymmetrical  but 


ACUTE  ATROPHIC  PARALYSIS. 


407 


I  <  - 


corresponding  distortion  of  the  feet.  This  affords  an  illustration  of 
another  fact,  namely,  that  when  there  is  a  partial  affection  of  both  legs 
the  paralysis  may  be  unsymmetrical  in 
the  two.  Much  less  commonly  the  calf 
muscles  suffer  more  than  the  others,  and 
there  results  talipes  calcaneus,  as  in  the 
case  shown  in  Fig.  113,  and  the  intrinsic 
muscles  of  the  foot  often  suffer  with 
those  of  the  leg.  In  the  thigh,  the  ex- 
tensors of  the  knee  are  affected  more 
frequently  than  the  flexors,  and  hence 
flexor  contraction  is  Tery  common,  and 
may  even  cause  subluxation  of  the  joint. 
The  flexors  of  the  hip  often  suffer, 
generally  with  the  extensors  of  the  kaee. 
Less  commonly  the  glutei  are  involyed, 
but  never  alone. 

In  the  arm,  almost  all  the  muscles  are 
sometimes  affected,  as  in  the  case  shown 
in  Fig.  114,  but  all  are  seldom  entirely 
paralysed.  The  intrinsic  muscles  of  the 
hand  often  suffer;  either  the  thenar 
muscles  or  the  interossei  may  be  most 
damaged.  An  instance  of  such  para- 
lysis of  the  interossei  is  shown  in 
Fig.  115.  The  forearm  muscles  are 
frequently  affected,  but  the  supinators 
may  escape  when  the  other  muscles  are 
involved.  The  deltoid  suffers  more  fre- 
quently than  any  other  single  muscle  of 
the  arm.  It  may  be  paralysed  alone  or  in 
association  with  other  muscles  ;  occasion- 
ally the  deltoid,  supra-  and  infra- spinatus, 
biceps,  and  supinators  are  all  involved  in  the  "  upper  arm  type  "  of 
palsy  of  Erb*  (see  p.  111).     But  other  muscles  than  these  may  be 


/ 


; 


Fjg.  114. — Atropliio  spinal  para- 
lysis, infantile  form.  Wasting 
of  all  the  muscles  of  the  left 
arm  from  the  deltoid  down- 
wards, and  of  the  right  ser- 
ratus  magnus. 


Fig.  115. — Atrophic  spinal  paralysis  (adult  form)  Mffccting  the  interossei 
and  thenar  muscles,  and  also  the  deltoid. 


•  See  a  paper  by  Dr.  Beevor,  '  Med.-Chir.  Trans.,'  1885. 


408  SPINAL    CORD. 

associated  with.  tLe  deltoid.  The  irregularity  of  tlie  grouping  is  sliown 
by  the  fact  that  the  triceps  is  often  affected  with  these  muscles. 
In  the  case  shown  in  Fig.  115  the  deltoid  and  intrinsic  muscles  of  the 
hand  were  wasted,  and  no'  other  muscles.  The  serratus  magnus  is 
occasionally  affected  as  in  Fig.  114;  in  this  case,  although  the  left 
arm  had  suffered  severely,  as  the  figure  shows,  on  the  right  side  only 
the  serratus  was  involved.  With  the  serratus,  the  upper  part  of  the 
pectoralis  major  is  sometimes  associated  in  paralysis  (as  it  is  in 
normal  function,  see  p.  37),  the  lower  part  being  unaffected;  it  was  so 
in  this  patient.  The  middle  part  of  the  trapezius  and  other  scapular 
muscles  are  occasionally  involved.  The  neck  muscles  rarely  suffer,  but 
the  diaphragm  is  occasionally  paralysed.  Although  the  intercostals 
and  other  trunk  muscles  are  so  often  weakened  in  the  early  stage,  con- 
siderable permanent  atrophy  is  very  rare.  Curvature  of  the  spine  is 
sometimes  produced  in  consequence  of  the  patient  being  allowed  to 
sit  up  while  the  muscles  are  weak.  It  may  be  a  lateral  or  an  antero- 
posterior curve,  according  as  the  weakness  is  on  one  or  both  sides.  I 
have  met  with  considerable  depression  of  the  lower  part  of  the  wall 
of  the  thorax  on  the  left  side  from  paralysis  of  the  intercostal 
muscles  at  the  spot,  perhaps  combined  with  hindered  growth  of  the 
ribs.  The  muscles  supplied  by  cranial  nerves  are  scarcely  ever 
affected.  I  have  once  seen  complete  paralysis  of  one  side  of  the  face, 
associated  with  wasting  in  the  limbs,  in  an  otherwise  characteristic 
case,  and  an  instance  of  affection  of  the  face  and  tongue  has  been, 
recorded  by  Pasteur.* 

The  Course  of  the  disease  has  been  already  sketched.  There  are 
(1)  an  initial  stage  of  paralysis,  rapidly  increasing,  often  accompanied 
with  fever  ;  the  duration  of  this  stage  is  from  a  few  hours  to  a  week 
or  even  more ;  (2)  a  stationary  period,  which  lasts  for  a  week  to  a 
month ;  (3)  a  stage  of  "  regression,"  during  which  the  palsy  passes 
away,  except  from  certain  parts  in  which  wasting  occurs ;  this 
regressive  period  usually  occupies  from  one  to  six  months ;  (4) 
a  chronic  stage  (overlapping  the  last),  during  which  atrophy  con- 
tinues ;  slight  improvement  may  occur,  but  contractures  and  de- 
formities are  developed.  The  duration  of  this  stage  is  indefinite, 
because,  wherever  muscular  tissue  remains  and  some  voluntary  power 
returns,  this  slowly  improves,  by  increased  development  of  the  muscle 
under  the  influence  of  use,  and  this  continues  in  slight  degree  for 
years.  This  gain  is  often,  however,  more  than  counterbalanced  by 
th.e  interference  with  growth,  and  by  the  occurrence  of  deformities. 
Even  in  the  slightest  cases  complete  recovery  is  extremely  rare,  and 
the  limb  remains  smaller  than  its  fellow.  On  the  other  hand,  death 
from  the  disease  is  equally  uncommon,  and  occurs  chiefly  in  the 
early  stage.     It  is  not  improbable  that  some  children  die  from  the 

•  '  Lancet,'  1887,  vol.  ii.  It  is  probable  that  when  the  cranial  nerve  nuclei  are 
affected,  the  vital  centres  become  involved  in  most  instances,  so  that  the  patients  do 
not  survive. 


ACUTE  ATEOrHin  PAEALTSIS.  409 

initial  disturbance  before  its  nature  is  recognised.  Now  and  then 
death  occurs  at  the  end  of  the  first  weet  or  ten  days,  from  universal 
paralysis  or  from  some  such  severe  cerebral  disturbance  as  existed  in 
the  cases  mentioned  above,  or  as  followed  the  spinal  lesion  in  a  case 
in  which  paralysis  in  the  arms  came  on  upon  the  lliird  day  of  the 
initial  fever  and  reached  its  height  on  the  fifth  day,  farad ic  irrita- 
bility being  lost  by  the  seventh  in  many  muscles  of  the  left  arm ;  a 
little  rigidity  of  the  neck  then  occurred,  and  on  the  tenth  day  the 
patient  began  to  vomit,  complained  of  increased  headache,  and  had 
hallucinations ;  on  the  eleventh  day  he  became  comatose  and  died. 
Bronchial  catarrh  is  an  occasional  cause  of  death,  when  the  respii-a- 
tory  muscles  are  weak. 

Eelapses  are  very  rare,  and  occur  chiefly  in  the  form  of  a  separate 
later  extension  of  the  disease.  Second  attacks  are  almost  unknown ; 
one  instance  only  was  met  with  in  the  series  of  214  cases. 

Sequelae,  beyond  those  mentioned  as  part  of  the  symptoms  of  the 
disease,  are  also  very  rare.  As  a  rule  the  general  and  nervous  health 
of  the  patients,  and  the  duration  of  life,  are  uninfluenced  by  the  local 
palsy.  In  a  few  cases,  however,  some  other  chronic  affection  of  the 
spinal  cord  has  come  on  when  the  subjects  of  infantile  paralysis  have 
reached  adult  life.  Progressive  muscular  atrophy  has  been  several 
times  observed  to  start  from  the  paralysed  limb,  and  even  to  be 
apparently  excited  by  a  fracture  of  the  bone.*  Acute  and  subacute 
polio-myelitis  in  adult  life  have  also  been  observed  as  a  sequel,  and  I 
have  twice  seen  the  symptoms  of  lateral  sclerosis  slowly  developed,  in 
one  case  at  seventeen,  in  the  other  at  twenty-eight.  In  the  spinal 
cord  of  the  subject  of  old  infantile  paralysis  there  seems  thus  to 
persist  some  disposition,  slight  though  it  be,  to  fresh  disease,  and  the 
cases  of  lateral  sclerosis  mentioned  show  that  the  liability  to  disease 
is  not  limited,  as  has  been  thought,  to  the  grey  matter.f  The  altered 
structure  of  the  bones  has  been  known  to  facilitate  their  fracture ;  in 
one  case  the  bones  in  the  affected  limb  were  broken  on  three  separate 
occasions  (Dutil). 

PATHOLOGiCAii  Akatomy.— For  a  long  time,  infantile  paralysis  was 
believed  to  be  a  peripheral  affection,  having  its  seat  in  the  muscles.  But 
improved  methods  of  examining  the  nerve-centres  have  shown  changes 
in  the  spinal  cord  which  have  the  characters  of  an  acute  inflammation 
of  the  grey  substance  of  the  anterior  horns.  The  correspondence 
between  the  infantile  and  adult  forms  makes  it  certain  that  the  latter 
are,  in  most  instances,  of  the  same  nature.  Subacute  atrophic  para- 
lysis in  adults  has  been  found,  however,  to  depend  on  disease  of  the 
nerves  in  some  forms  that  were  thought  to  be  spinal,  but  in  these  the 
palsy  is  strictly  symmetrical,  and  when  it  is  irregular  in  distribution 
the  disease  is  certainly  identical  with  the  infantile  form. 

Observations  on  the  changes  in  the  spinal  cord  in  the  early  stage 

•  Raymond,  'Prog.  Med.,'  1889  ;  Dutil,  'Gaz.  .Med.  de  Paris,'  1881. 

t  Compare  Ballet  and  Dutil,  'lievue  de  Medecine,'  Jauuarj,  1884,  (i.  18. 


410 


SPINAL    CORD. 


■^^T^'^vV-ffi^-,^ 


are  few.  A  case  recorded  by  Dr.  D.  Drummond,  in  all  probability 
one  of  tHs  disease,  is  by  far  tbe  earliest  on  record,  and  is  indeed  the 
earliest  possible.*  A  child,  five  years  old,  died  after  a  few  hours' 
acute  illness.  The  spinal  cord,  in  the  region  of  the  fourth  and  fifth 
cervical  nerves,  presented  undue  redness  of  the  anterior  grey  matter. 
The  vessels  running  from  the  surface  to  the  cornu  were  distended 
with   blood.     The  microscope  showed  distension  of  capillaries   and 

minute  extravasations  in  the 
grey  substance,  swelling  of 
the  neuroglial  elements  and 
of  the  ganglion-cells,  which 
were  granular  with  indistinct 
processes.  Another  important 
early  observation  is  that  of 
Dr.  Charlewood  Turner,t  six 
weeks  after  the  onset  (Fig. 
116).  In  this  and  other 
somewhat  later  cases,  acute 
changes  have  been  found  in 
the  anterior  cornua,  more 
advanced  than  in  Drum- 
mond's  case.  They  are  often 
widely  spread  in  slight  de- 
gree, and  attain  considerable 
intensity  in  one  or  more  foci, 
usually  in  the  cervical  or 
lumbar  enlargements  or  both. 
At  these  spots  the  anterior 
horn  is  softened ;  sometimes 
there  is  hsemorrhagic  infiltra- 
tion (Fig.  117),  sometimes  an 
actual  cavity  (Fig.  116,  A,  b). 
The  microscope  shows  extra- 
vasated  blood  often  massed 
along  the  vessels  (Fig.  116, 
c),  and  scattered  through  the 
grey  matter  with  other  cells 
such  as  are  met  with  in  mye- 
litis. There  are  also  granule 
corpuscles  and  other  products 
of  degeneration  of  the  nerve- 
elements.  These  elements, 
and  especially  the  motor 
nerve-cells,  have  almost  entirely  disappeared.  Where  the  change  is 
slighter  in  degree  there  is  a  leucocytal  infiltration,  a  few  granule 
corpuscles  are  seen,  while  the  nerve-cells  may  be  structurally  intact, 

*  '  Brain,'  April,  1885.  f  '  Path.  Trans.,'  vol.  xxv,  p.  £03. 


«% 


Fig.  116. — Acute  anterior  polio-myelitis  in  a 
child  2^  years  old,  six  weeks  after  the  onset. 
(After  Charlewood  Turner.)  A.  Section 
through  the  lowest  part  of  the  lumbar  en- 
largement, showinsr  a  cavity  visible  to  the 
naked  eye,  on  tlie  lett  side.  B.  Lef  i  ante- 
rior half  of  the  cord  under  a  low  magnify- 
ing power,  showing  destruction  of  almost 
the  whole  anterior  cornu.  c.  Portion  of 
anterior  cornu  in  the  upper  part  of  the 
lumbar  enlargement.  Numerous  corpuscles 
lie  in  thegninuliir  protoplasm  and  reticulum 
of  fine  fibres.  A  small  artery  is  encrusted 
with  several  layers  of  corpuscles  and  nuclei. 


ACUTE  ATROPHIC  PAEALYSIS. 


411 


but  swollen  and   granular.     Tlie   morbid   appearances  may  be  con- 
fined to  the  anterior  cornua,  or  may  extend  in  sKghter  degree  into 


-^>-t^< 


Fig.  117. — Acute  anterior  polio-myelitis  in  a  child  14  months  old.  Dr. 
F.  E.  Batten's  case.  The  affection  coimnenced  in  the  lees,  but  there 
were  electrical  changes  and  paralysis  in  the  arms  as  well.  The  child 
died  suddenly  on  the  fourteenth  day  of  the  illness.  The  section  made 
and  lent  by  Dr.  Batfen  shows  marked  engorgement  of  vessels,  with 
occasionally  actual  extravasation,  small-celled  infiltration,  and  changes 
in  the  anterior  horn  cells. 


the  adjacent  lateral  column,  in  which  the  nerve-fibres  near  the  gr^ 
matter  may  undergo   destructive   degeneration,  and   other   signs  of 


412 


SPINAL   CORD. 


inflammation  maj  be  seen.  Tlie  posterior  columns  are  always  un- 
affected. In  very  severe  cases  there  may  be  signs  of  slight  local 
meningitis  corresponding  in  position  to  the  region  chiefly  affected. 
This  region  corresponds  to  the  origin  of  the  nerves  supplying  the 
muscles  in  which  there  is  most  wasting,  and  the  anterior  nerve-roota 
which  arise  from  this  part  present  the  signs  of  acute  degeneration. 

Such  changes  indicate  a  general  or  interstitial  inflammation  of  the 
grey  matter.  But  some  observations  have  revealed  only  alterations 
in  the  motor  nerve-cells,  which  have  presented  granular  degeneration, 
the  matrix  between  them  being  quite  unaffected.*  This  suggests  that 
there  may  be  a  form  of  polio-myelitis  in  which  the  changes  are 
essentially  parenchymatous  and  not  interstitial. 

At  a  later  period,  years  after  the  onset,  the  appearances  presented 
accord  with  those  described  as  commonly  found  in  the  early  stage, 
allowance  being  made  for  the  difference  in  time.  The  anterior  cornu 
at  one  or  more  places  is  shrunken  (Fig.  118),  and  in  part  stains 


Fl&.  118.— Atrophic  spinal  (infantile)  paralysis.  A.  First  lumbar;  the  left  anterior 
cornu  is  smaller  than  the  right,  and  its  ganglion-cells  have  disappeared  with  the 
exception  of  the  inuer  group.  B.  Fourth  lumhar;  the  whole  left  half  of  the  cord 
is  smaller  than  the  other,  in  consequence  of  the  diminution  in  size  of  the  ant. 
horn.  Of  the  ganglion-cells  only  a  few  remain,  belonging  to  the  an tero- lateral 
group.     Similar  changes  existed  throughout  the  lumhar  enlargement.f 

deeply  with  carmine,  in  consequence  of  its  nervous  structures  being 
replaced  by  connective  tissue,  in  part  less  deeply  in  consequence  of 
"granular  disintegration"  of  the  substance.  The  motor  nerve-cells 
are  absent,  partially  or  entirely.  Sometimes  a  few  shrivelled  cells 
remain,  sometimes  one  or  more  groups  may  be  unaffected ;  or  one  or 
two  nerve-cells  of  normal  appearance  may  alone  remain  (see  Fig.  118). 
Small  foci  of  disease  may  be  seen  in  the  unaffected  part  of  the 
grey  matter,  and  in  these  the  motor  cells  may  be  smaller  than 
normal ;  the  intervening  plexus  may  have  perished.  When  one  horn 
is  much  damaged,  the  other  may  merely  present  fewer  nerve-cells  than 

*  Rissler, '  Nord.  Med.  Ark.,'  xx. 

t  For  these  sections  I  am  indebted  to  Dr.  H.  Humphreys,  of  St.  Leonards.  The 
paralysis  came  on  at  one  and  a  half  years  of  age,  two  years  before  death,  and  involved 
most  of  the  muscles  of  the  lelt  leg,  those  of  the  calf  in  greatest  degree,  so  that 
talipes  calcaneus  had  developed. 


ACUTE  ATROPHIO  PARALYSIS.  413 

normal,  with,  perhaps  a  little  condensation  of  the  interstitial  tissue. 
The  antero-lateral  column  is  usually  lessened  in  size,  and  may  present 
slight  sclerotic  changes,  especially  in  a  zone  contiguous  to  the  grey 
substance,  whence  thickened  septa  may  radiate  into  the  white  column. 
This  is  apparently  due  to  the  extension  of  an  intense  inflammation 
of  the  grey  substance  to  the  adjacent  part  of  the  white  column. 
Sometimes  a  slighter  sclerosis  can  be  traced  through  the  lateral 
column ;  this  is  seen  chiefl.y  in  cases  of  long  duration,  and  is  probably 
secondary.  With  extreme  damage  to  both  cornua  there  may  be 
ultimately  great  wasting  of  the  white  columns  on  each  side.*  In 
consequence  of  these  alterations,  the  affected  half  of  the  cord  is 
conspicuously  smaller  than  the  other,  even  to  the  naked  eye,  and  the 
difference  is  greatest  where  the  damage  to  the  grey  matter  is  most 
intense.  The  anterior  nerve-roots  at  the  most  affected  part  are 
small  and  grey,  and  the  degeneration  of  the  motor  fibres  may  be 
traced  down  the  nerve-trunks.  Often  a  few  fibres  present  a  normal 
appearance  although  the  rest  have  perished ;  possibly  from  the  sym- 
pathetic system. 

The  muscular  fibres,  in  early  cases,  have  been  found  narrower  than 
normal,  and  in  a  state  of  granular  degeneration,  with  an  increase  in 
the  nuclei  of  the  sheath  and  of  the  interstitial  tissue.  Grannies  and 
pigment  masses  accumulate  between  the  sarcolemma  sheaths.  In 
extreme  cases  (those  in  which  all  electrical  excitability  is  early 
lost)  the  degeneration  proceeds  to  complete  disappearance  of  the 
fibres,  the  place  of  which  is  occupied  by  fibrous  tracts,  developed 
partly  from  the  sarcolemma  sheaths  and  partly  from  the  interstitial 
connective  tissue.  It  is  common  to  find,  here  and  there,  a  fibre 
presenting  normal  appearances.  Sometimes  fat  accumulates  in  the 
interstitial  tissue,  so  that  the  reduction  in  the  bulk  of  the  muscle 
may  be  less  than  that  of  the  fibres.  In  slighter  cases,  in  which 
partial  recoveiy  takes  place,  some  of  the  fibres  probably  regain  their 
normal  appearance,  even  though  they  have  undergone  slight  granular 
degeneration.  Usually,  however,  many  fibres  perish ;  those  which 
recover  are  smaller  than  normal,  and  the  original  volume  of  the 
muscle  is  rarely  regained.  Although  there  may  be  a  permanent  increase 
in  the  interstitial  tissue,  this  undergoes  contraction  and  comes  to 
occupy  but  a  small  bulk.  Muscular  fibres  are  often  seen  that  are 
larger  than  normal,  and  this  is  not  (as  has  been  thought)  a  transient 
condition,  for  it  has  been  observed  seventy  and  forty-five  years  after 
the  onset.f    It  is  perhaps  sometimes  a  true  hypertrophy.^ 

The  bones  have  been  found  not  only  shorter  than  normal,  but  also 

•  See  •  British  Med.  Journal,'  1887,  p.  187. 

t  Joffroy  and  Achard,  'Arch,  de  Med.  exp.,'  1889;  Dejerine  and  Huet,  'Arch. 
de  Phys.,'  1888. 

X  Dejerine.  It  is  said  to  have  heen  met  with  in  muscles  totally  paralysed ;  but 
it  is  doubtful  whether  **  total  palsy  "  of  a  muscle  excludes  voluntary  contraction  of 
some  fibres  in  it. 


*l4f  SPINAL    COED. 

smaller,  vrith.  their  angles  rounded,  tlie  compact  substance  more 
uniform,  and  tlie  Haversian  canals  smaller  (Jofcoy  and  Achard), 

The  brain  bas  been  almost  always  found  normal.  In  one  case  with 
extensive  paralysis,  of  long  duration,  some  atrophy  was  found  in  the 
motor  region  of  the  cortex  (Sanders).  It  is  probable  that  some 
acute  changes  would  be  found  in  cases  that  die  with  the  cerebral  sym- 
ptoms already  described. 

No  observations  have  as  yet  been  made  on  the  acute  primary  poly- 
neuritis that  seems  occasionally  to  coincide  with  the  inflammation  of 
the  cord,  perhaps  sometimes  to  exist  alone  without  any  cord  affection.* 
It  is  probable  that,  in  the  cases  in  which  the  nerves  are  tender  in  the 
middle  of  their  course,  the  sheath  will  be  found  inflamed,  and  only 
the  motor  fibres  will  present  such  signs  of  secondary  degeneration  as 
always  result  from  the  lesion  of  the  cord. 

Pathologt. — Both  the  character  of  the  lesion  and  its  mode  of 
onset  suggest  that  it  is  inflammatory  in  the  majority  of  cases.  In  rare 
instances,  as  already  stated,  the  almost  instantaneous  onset  makes 
it  probable  that  the  lesion  is  different  in  character,  is  vascular  in 
its  nature,  probably  haemorrhage.  It  is  likely  that  these  cases  are 
really  haemorrhagic  myelitis,  in  which  the  usual  initial  vascular  dis- 
turbance is  so  severe  as  to  produce  an  extravasation.  Signs  of  inflam- 
mation, do  not  precede  the  sudden  onset. 

The  symptoms  are  well  explained  by  the  pathological  facts  that 
have  been  ascertained,  extended  by  the  simple  inferences  that  are 
indicated  by  the  symptoms.  The  lesion  interrupts  the  motor  path, 
causing  immediate  palsy,  which  results  from  damage  to  the  cells  and 
nerve-plexus  of  the  grey  matter,  whether  slight  or  severe.  The 
recovery  of  the  slightly  damaged  structures  restores  the  voluntary 
path,  and  even  if  the  fibres  from  the  cells  degenerate,  they  seem  to 
regain  normal  nutrition  by  regeneration  when  the  cells  recover.  But 
when  the  motor  cells  are  destroyed  the  fibres  from  these  degene- 
rate permanently.  As  the  slighter  damage  is  usually  far  wider  than  the 
destruction,  the  initial  palsy  is  more  extensive  than  the  persistent 
atrophy,  and  the  extent  of  such  is  purely  a  matter  of  this  relative 
area  of  trifling  and  severe  degree  of  lesion.  Nerve-cells  that  are 
destroyed  are  never  renewed,  and  hence  the  permanence  of  the  muscular 
wasting  that  dej)ends  on  such  destruction.  When  a  few  nerve-cells 
escape  destruction,  a  few  nerve-fibres  recover  and  a  little  muscle-tissue 
remains,  too  slight,  it  may  be,  to  be  of  use.  The  occasional  interference 
with  the  conducting  tracts  in  the  white  columns  is  explained  by  the 
obvious  extension  of  the  inflammation,  which,  when  very  intense,  may 
even  spread  through  all  the  structures  of  the  cord,  as  in  the  rare  cases 
in  which  sensation,  as  well  as  motion,  is  lost.  But  such  cases,  which 
are  most  rare,  differ  from  true  transverse  myelitis  in  the  rapidity  with 

*  As  was  probably  the  case  in  two  cases  of  paralysis  in  children  shown  to  the 
Harveian  Society  of  London  by  Dr.  Leonard  GuiLrie,  'Lancet,'  1897,  voL  i,  p.  448. 


ACUTE  ATKOFHIO  PAIULYSIS.  415 

wliich  tlie  damage  restricts  itself  to  its  special  seat,  and  sensory  con- 
duction is  restored.  The  fact  that  the  disease  of  the  sensory  tracts  is 
only  secondary  and  trifling,  is  emi:)hasised  by  the  fact  that  even  in  these 
cases  th«re  are  never  the  acute  bedsores  that  would  surely  ensue  in  a 
case  of  true  transverse  or  total  myelitis  of  corresponding  seat. 

The  observations  mentioned  on  p.  412  suggest  that  there  are  two 
varieties  of  the  inflammation  of  the  grey  matter,  such  as  we  have 
recognised,  in  myelitis  generally;  a  parenchymatous  inflammation 
consisting  in  a  primary  change  in  the  nerve-cells,  and  an  interstitial 
or  general  inflammation,  as  in  the  common  forms  of  myelitis,  in- 
volving the  nerve-cells  only  as  one  of  many  structures  equally  damaged 
by  a  process  that  has  no  special  relation  to  the  nerve-elements.  The 
distinction  is  important,  because  we  should  expect  to  find,  in  the 
former  cases,  a  wider  slight  initial  affection  than  in  the  others,  and 
finally  a  more  definite  relation  to  function  in  the  parts  diseased. 

The  true  pathology  of  the  malady,  its  relation  to  its  causes,  is  still 
obscure.  The  one  salient  fact  that  we  can  discern,  though  scarcely 
define,  is  the  evidence  of  a  blood- state  afforded  by  the  general  sym- 
ptoms. That  these  symptoms  are  not  the  consequence  of  the  local 
inflammation  is  shown  by  their  disparity  in  degree,  and  difference  in 
time.  It  is  probable,  therefore,  that  the  spinal  lesion  is  not  the  cause 
of  the  constitutional  disturbance,  but  is  rather  an  effect  of  the  cause  of 
the  latter.  It  seems  impossible  otherwise  to  understand  the  extreme 
variation  in  the  two  forms  of  disturbance.  But  we  have  no  evidence 
as  to  the  nature  of  the  blood-state,  and  there  are  no  other  effects, 
commonly  associated  with  the  myelitis,  to  indicate  the  general  patho- 
logical tendency  of  its  cause.  The  closest  analogy  is  with  some  forms 
of  neui'itis,  and  this,  as  we  have  seen,  may  co-exist  with  the  spinal 
lesion,  as  a  consequence,  at  any  rate,  of  exposure  to  cold.  Indeed,  it 
is  probable  that  the  inflammation  of  the  nerves  is  the  chief  or  even  the 
only  morbid  process  in  some  of  these  cases.  On  the  other  hand,  it 
must  be  noted  that  in  the  epidemics  of  the  disease,  there  were,  besides 
cases  of  spinal  cord  disease,  simultaneous  cases  in  the  same  places  in 
which  the  evidence  pointed  to  an  analogous  inflammation  in  the  cere- 
brum. In  one  epidemic,  the  American  one,  the  lower  animals  also 
suffered  (see  p.  416) .  The  variations,  alike  in  the  general  symptoms  and 
in  the  inflammation  of  the  cord,  and  especially  the  indications  that  the 
latter  may  be  either  parenchymatous  or  interstitial,  suggest  that  the 
causal  blood-state  also  varies  in  different  cases.  We  have  no  indication 
of  the  way  in  which  the  remarkable  relation  to  season  is  produced, 
whether  by  predisposing  the  individual  or  favouring  the  development 
of  some  toxic  agent.  We  must,  however,  recognise  as  an  essential 
element  the  predisposing  influence  of  age,  which  we  may  associate 
with  the  facts  that  the  structural  development  of  the  nervous  system 
is  complete,  but  the  function  of  the  nerve-elements  must  lack  the 
stability  that  comes  only  from  continued  use,  and  that  the  period  is 
generally  that  which  coincides  with  the  first  serious  demand  on  the 


416  SPINAL  CORD. 

functional  energy  of  tlie  grey  matter  of  the  cord.  We  may  remember 
also  the  vascular  activity  that  all  function  entails,  and  the  readiness 
with  which  the  vaso-motor  system  of  children  is  disturbed.  Lastly 
the  occurrence  of  the  disease  in  more  than  one  member  of  the  same 
family  indicates  a  congenital  disposition  of  the  system  to  react  in  a 
similar  manner  to  certain  external  agencies,  while  a  special  feature  in 
the  latter  may  perhaps  explain  the  occasional  epidemic  character  of  a 
disease,  and  furnish  grounds  for  ascribing  to  it  an  "infective" 
character.*  The  evidence  available  suggests  that  it  is  probably  due  to 
some  chemical  change  in  the  blood  analogous  to  that  which  seems  to 
cause  rheumatic  fever,  though  probably  distinct  from  it — a  change 
excited  by  cold,  disposed  to  by  the  effect  of  heat,  the  result  of  some 
derangement .  of  metabolism  which  we  cannot  yet  understand,  and 
perhaps  having  underlying  these  effects  some  organismal  cause.  This 
hypothesis  does  not  exclude  the  action  of  traumatic  influences  in 
determining  the  disease.  Such  a  glimpse  of  its  pathological  relations 
is  all  that  we  can  at  present  obtain. 

Diagnosis. — This  rarely  presents  any  difficulty  except  in  the  early 
stage.  When  the  initial  paralysis  is  passing  away,  and  the  wasting  is 
distinct,  the  nature  of  the  case  is  sufficiently  evident,  and  is  cor- 
roborated by  the  electrical  reactions,  by  the  loss  of  reflex  action,  and 
by  the  absence  of  any  impairment  of  sensibility.  At  the  onset, 
however,  the  symptoms  may  readily  be  misinterpreted.  The  most 
common  error  is  to  attach  too  much  importance  to  vomiting,  and 
to  regard  the  attack  as  simply  gastric.  G-eneral  disturbance  is 
naturally  ascribed  to  some  general  cause  "until  nervous  symptoms 
manifest  themselves.  Even  then  there  is  risk  of  error.  On  the  one 
hand,  as  we  have  seen,  a  diagnosis  of  a  general  disease  is  not  always 
relinquished  when  paralysis  appears,  but  the  latter  is  regarded  as 
secondary.  On  the  other  hand,  especially  in  young  children,  the 
existence  of  paralysis  is  often  overlooked  at  first,  and  it  is  supposed 
that  the  child  doi  s  not  move  because  it  is  prostrate.  This  error  will 
not  be  made  if  it  is  remembered  that  mere  prostration  seldom  causes 
total  immobility,  and  a  fortiori  it  does  not  produce  local  immobility. 
When  the  pyrexia  ceases,  and  the  loss  of  power  persists  and  increases, 
the  existence  of  paralysis  is  always  unmistakeable.  The  initial  general 
disturbance  must  then  be  regarded  as  part  of  the  disease  unless  there 
is  the  clearest  evidence  of  its  independent  nature. 

In  adults,  the  danger  of  mistaking  paralysis  for  prostration  is  con- 
siderably less,  but,  on  the  other  hand,  the  general  symptoms  are  as 
likely  to  be  misinterpreted  as  in  the  case  of  children.  The  rheuma- 
toid pains,  which  are  so  common,  are  usually  regarded  as  evidence  of 
acute  rheumatism,  especially  when  the  affection  follows  exposure  to 
cold.     Whenever   rheumatoid  pains  are  not  localised  in  the   joints 

•  See  Medin,  '  Neur.  Cent.,'  1896,  p.  1120 ;  Buccelli,  '  Neur.  Cent.,'  1897,  p.  798  ; 
Sowerby,  '  Journ.  Am.  Med.  Assoc.,'  January,  1896;  Pasteur,  *  Trans.  Clin.  Soc.,* 

1897. 


ACUTE  ATROPHIC  PAEALYSIS.  417 

and  especially  wlien  they  are  spontaneous,  and  not  influenced  by  move- 
ment, tlie  possibility  of  tbeir  spinal  (or  nerve)  origin  should  be  remem- 
bered ;  and  other  indications  of  spinal  mischief,  such  as  local  loss  of 
power,  tingling  or  formication,  should  be  carefully  watched  for  and 
receive  due  weight. 

Of  other  spinal  cord  diseases,  all  chronic  lesions  are  distinguished 
at  once  by  the  onset.  From  other  acute  diseases,  moreover,  the  dis- 
tinction is  only  difficult  in  the  early  stage.  As  soon  as  the  initial 
palsy  begins  to  lessen,  and  the  muscles  in  one  part  lose  faradic  irrita- 
bility and  begin  to  waste,  the  nature  of  the  case  admits  of  no  doubt. 
The  diagnosis  may  be  made  with  certainty  after  the  end  of  the 
first  week  by  the  electrical  reaction.  If  we  find  loss  of  faradic  irrita- 
bility, it  is  certain  that  the  characteristic  alteration  in  voltaic  irrita- 
bility will  follow.  The  isolated  induced  shocks  should  be  used  instead 
of  the  serial  "current,"  because  any  harmful  stimulation  of  the 
sensory  nerves  is  thereby  avoided.     (See  Diseases  of  the  Nerves.) 

Acute  transverse  myelitis  is  only  simulated  when  the  inflammation 
of  the  grey  matter  is  bilateral  and  so  intense  as  to  extend  to  the 
white  columns,  but  the  age  of  the  patient  generally  suggests  correctly 
the  nature  of  the  disease.  The  myelitis  of  childhood  is  polio-myelitis. 
The  pseudo-transverse  variety — polio-myelitis,  transverse  through  its 
intensity — is  seated  in  one  of  the  enlargements,  usually  the  lumbar ; 
the  true  transverse  form  usually  occurs  in  the  dorsal  region.  Eeal 
difficulty  is  confined  to  adults,  in  whom  there  is  extensive  cervical  or 
lumbar  myelitis  involving  the  grey  matter.  In  young  adults,  polio- 
myelitis is  more  likely ;  this  is  indicated  also  by  what  is  best  described 
as  a  regressive  onset  in  contrast  to  one  that  is  progressive.  In  the 
former  a  wide  extent  is  quickly  reached,  and  the  symptoms  then  tend 
to  lessen ;  in  the  latter  the  morbid  process  is  a  longer  time  reaching 
its  height :  in  the  former  the  symptoms  clearly  show  that  the  most 
severe  affection  is  of  the  anterior  grey  matter ;  in  the  latter,  severe 
anaesthesia  or  trophic  disturbance  usually  points  to  an  equally  intense 
lesion  of  the  other  parts  of  the  cord. 

The  separation  of  subacute  from  acute  myelitis  of  the  grey  substance 
is,  to  a  large  extent,  arbitrary.  The  symptoms  are  similar,  but  the 
onset  is  less  rapid  and  occupies  more  than  a  week.  The  distinction  of 
multiple  neuritis  is  from  the  subacute  rather  than  the  acute  spinal 
atrophies,  and  is  considered  elsewhere. 

The  diagnosis  from  paralysis  of  cerebral  origin  is  usually  easy.  In 
cerebral  palsy  there  is  never  loss  of  faradic  irritability  or  extreme 
muscular  wasting,  or  loss  of  the  muscle-reflex  action.  In  the  spinal 
affection  there  is  no  trace  of  the  mobile  spasm  that  is  common  after 
infantile  hemiplegia.  Any  cerebral  symptoms  which  may  attend  the 
onset  of  polio-myelitis  are  subordinate  in  significance  to  the  state  of 
the  muscles  as  evidence  of  the  permanent  lesion.  Convulsions  at  the 
onset  of  infantile  spinal  paralysis  are  general ;  those  that  result  from 
a  cerebral  lesion  are  usually  unilateral  or  commence  locally.  The  chief 
VOL.  I.  27 


418  SPINAL   CORD. 

difficTilty  arises  wlien  arm  and  leg  are  inTolved  on  the  same  side. 
These  cases  are  rare,  and  present  no  difficulty  if  attention  is  paid  to 
the  nutrition  and  reaction  of  the  muscles. 

The  exceedingly  slow  onset  of  pseudo-hypertrophic  paralysis, 
developing  gradually,  as  it  does,  with  the  child's  growth,  ought  to 
render  its  confusion  with  polio-myelitis  impossible.  A  reasonable 
doubt  can  only  arise  in  a  slight  case  in  which  the  onset  was  unnoticed 
or  forgotten.  I  have  once  tnown  slight  atrophic  palsy  of  the  exten- 
sors of  the  knee  to  induce  the  habit  of  putting  the  hand  on  the  knee 
in  rising  from  the  ground — an  action  often  thought  to  be  peculiar  to 
the  pseudo  hypertrophic  disease,  but  acquired  when  weakness  of  the 
extensors  occurs  in  early  childhood  from  any  cause. 

Diseases  outside  the  nervous  system  which  have  been  mistaken  for 
infantile  paralysis  are  chiefly  those  in  which  local  pain  interferes  with 
the  movement  of  the  limbs,  and  the  child  is  young.  I  have  known, 
for  instance,  the  mistake  to  be  made  in  the  case  of  hip-joint "  disease, 
necrosis  of  the  femur,  and  the  affection  termed  "  scorbutic  rickets,"  in 
which  there  is  enlargement  of  the  shafts  of  the  long  bones,  extreme 
pain  on  movement,  and  spongy  gums.  In  all  these  cases  a  careful 
examination  will  show  that  movement  is  interfered  with  only  by  the 
pain ;  there  is  no  actual  paralysis,  and  there  is  no  interference  with 
reflex  action.  The  preservation  of  the  knee-jerk  is  often  of  great 
significance  ;  it  at  once  excludes  atrophic  palsy  in  any  case  in  which, 
the  extensors  of  the  knee  seem  feeble. 

The  points  above  described  should  always  enable  the  diagnosis  to  be 
made.  It  is  impracticable  to  enumerate  the  distinctions  from  every 
disease  with  which  confusion  is  possible,  for  experience  shows  that 
there  is  no  form  of  palsy  with  which  a  common  disease,  such  as  this, 
is  not  at  some  time  confounded. 

Peognosis. — The  danger  to  life  is  probably  greatest  when  there  is 
severe  constitutional  disturbance,  which  may  even  precede  the  develop- 
ment of  the  characteristic  paralytic  symptoms.  In  the  stage  of 
paralysis  there  is  peril  only  when  the  chief  disease  is  in  the  cervical 
region,  and  respiration  is  interfered  with;  but  this  danger  is  small 
imless  an  attack  of  bronchial  catarrh  is  intensified  by  the  palsy. 
Definite  cerebral  symptoms  involve  danger  proportioned  to  their 
character  and  degree,  but  it  is  generally  less  in  reality  than  in  appear- 
ance. In  the  vast  majority  of  cases  the  disease  involves  no  immediate 
danger  to  life.  But  children  are  left  with  little  power  of  resistance  to 
other  morbid  influences,  and  occasionally  succumb  to  some  other 
iUness,  as  an  acute  specific  disease,  or  an  attack  of  bronchitis,  a  few 
weeks  or  months  after  the  onset  of  the  paralysis. 

As  soon  as  the  paralysis  has  become  stationary,  i,  e.  has  not 
increased  for  twenty-four  hours,  the  danger  of  further  extension  is 
small.  But  the  question  at  once  arises,  and  is  anxiously  asked, — 
What  will  be  the  permanent  condition  ?  Will  there  be  lasting 
paralysis  ?     An  answer  cannot  be  given  until  the  end  of  the  first 


ACUTE  ATROPHIC  PARALYSIS,  419 

weeTc  or  ten  days,  and  tlien  only  by  means  of  an  electrical  examina- 
tion. Whatever  muscles,  at  tlie  end  of  that  time,  have  lost  faradic 
irritability  will  certainly  waste,  will  remain  for  a  long  time  paralysed, 
and  will  probably  be  permanently  affected  in  some  degree,  slight  or 
severe.  On  the  other  hand,  if  there  is  no  loss  of  irritability  at  the  end 
of  ten  days,  but  it  is  apparent  at  the  end  of  a  fortnight  or  three 
weeks,  the  wasting  will  be  slighter  in  degree,  and  some  ultimate 
recovery  may  be  anticipated  even  in  the  most  affected  part.  Where 
there  is  no  loss  of  irritability  the  paralysis  will  pass  away  in  the 
course  of  a  few  weeks,  or  at  most  of  a  few  months.  When  faradic 
irritability  is  lost  early  and  completely,  the  wasting  will  be  rapid  and 
great,  and  it  is  unlikely  that  there  wiU  be  much  recovery.  The 
return  of  faradic  irritability  that  has  been  lost  is  a  favorable  indica- 
tion; it  signifies  nerve-regeneration,  and  will  be  followed  by  an 
increase  in  voluntary  power.  Without  an  electrical  examination  it  is 
necessary  to  wait  longer  before  a  prognosis  can  be  given,  imtil  distinct 
wasting  on  the  one  hand,  or  improvement  on  the  other,  indicates  the 
regions  in  which  the  paralysis  will  persist  and  in  which  it  will  pass 
away.     Even  then  the  prognosis  cannot  be  so  definite. 

In  the  chronic  stage  the  prospect  of  ultimate  recovery  depends  on  the 
rate  at  which  wasting  developed,  on  the  electrical  reaction,  and  on  the 
duration  of  the  case.  Where  there  is  no  sign  of  returning  power  at  the 
end  of  three  months  very  little  recovery  will  occur;  the  nerve-ceUs 
are  destroyed,  their  renewal  is  impossible.  The  preservation  of 
voltaic  irritability  (that  of  the  muscular  fibres)  is  so  far  satisfactory 
that  it  shows  there  has  been  no  destructive  degeneration  of  the 
muscles ;  and  if  voluntary  power  is  increasing,  it  indicates  favorable 
conditions  for  its  exertion,  but  it  does  not  lessen  the  grave  significance 
of  persistent  palsy  and  the  absence  of  faradic  irritability,  which  indi- 
cate persistent  nerve-degeneration.  On  the  other  hand,  if,  at  the  end 
of  one  or  two  months,  some  faradic  irritability  can  be  still  detected, 
although  low  in  degree  (i.  e.  elicited  only  by  a  strong  current), 
improvement  is  probable,  and  may  become  considerable.  It  is  neces- 
sary, in  the  case  of  children,  to  remember,  and  it  is  important  to 
warn  the  friends,  that  the  growth  of  the  most  affected  limb  will  be 
hindered,  and  that  this,  ia  the  case  of  the  leg,  may  render  the  effect 
of  the  paralysis  more  obtrusive  by  its  interference  with  the  gait. 
Otherwise  they  are  distressed  by  what  seems  to  them  an  increase  ia  the 
disease,  but  is  really  compatible  with  continued  improvement. 

Treatment. — The  treatment  of  the  acute  stage  of  the  disease  is 
essentially  the  same  as  that  of  myelitis,  already  described,  and  the 
rules  and  principles  already  stated  need  not  be  here  repeated.  In  the 
initial  stage  it  should  be  that  of  the  general  state,  guided  by  any 
special  causal  indications  that  may  be  detected ;  such  as  free  sweating, 
followed  by  salicylate  of  soda  or  salicin,  in  a  case  distinctly  due 
to  exposure  to  cold.  We  may  hope  that  future  observations  will 
afford  us  some  indications  regarding  the  means  of  counteracting 


420  SPINAL    CORD. 

other  blood-states  that  apparently  exist  at  the  onset,  and  are  con- 
cerned in  the  production  of  the  lesion.  At  present  we  are  without 
this  help,  and  can  only  treat  the  initial  stage  as  we  should  any  other 
local  inflammation,  directing  our  treatment  to  the  symptoms  that 
may  be  present,  and  the  conditions  that  apparently  underlie  them. 
The  child  should  be  kept  at  perfect  rest,  on  the  side,  so  that  the  spine 
is  not  the  most  depending  part.  Warmth  may  be  applied  over  the 
affected  part  of  the  cord  by  poultices  or  fomentations.  The  marked 
relief  these  give,  when  there  is  spinal  pain,  make  it  probable  that  they 
exert  a  beneficial  influence  in  all  cases. 

In  such  a  disease — in  which  there  is  a  natural  tendency  for  the 
morbid  process  to  cease  to  spread,  and  then  to  lessen  in  extent — the 
difficulty  of  ascertaining  the  effect  of  treatment  on  the  lesion  of  the 
spinal  cord  is  very  great.  Full  doses  of  belladonna  or  ergot  have 
been  credited  with  the  control  and  arrest  of  the  morbid  process,  but 
only  on  the  evidence  of  a  coincidence  in  isolated  cases,  the  value  of 
which  is  small  in  a  disease  so  irregular  in  its  course.  Either  may  appa- 
rently be  employed,  however,  without  fear  of  harm.  But  probably  the 
measures  mentioned  above  directed  to  the  local  inflammation  have  far 
more  influence  than  drugs  on  the  morbid  process.  As  in  all  acute 
diseases  of  the  nervous  system,  the  chief  room  for  effective  treatment 
rests  with  the  practitioners  who  see  the  cases  in  the  early  stage. 

When  the  acute  onset  is  over  and  the  spinal  lesion  has  become 
stationary  the  careful  management  of  the  early  stage  should  be  con- 
tinued for  a  time,  because  a  relapse  or  recurrence,  however  rare,  is  not 
entirely  unknown.  Such  care  is  especially  needed  in  cases  in  which 
the  constitutional  disturbance  has  been  prolonged,  or  has  continued 
after  the  onset  of  the  spinal  symptoms,  or  in  which  the  latter  have 
come  on  in  distinct  stages.  Under  these  circumstances,  perfect  rest 
should  be  maintained  for  one  or  two  weeks  more.  The  same  prolonged 
care  is  needed  when  there  is  persistent  tenderness  in  the  limbs,  or  any 
indications  of  an  independent  neuritis.  Where  wasting  is  taking  place, 
some  slight  tenderness  of  the  nerves  and  of  the  muscles  must  be 
expected  to  accompany  the  process,  and  this,  being  purely  secondary 
in  nature,  does  not  call  for  special  treatment. 

When  the  acute  stage  is  over,  and  the  condition  of  the  patient  is 
stationary  or  improving,  tonics  are  generally  both  useful  and  needed, 
especially  iron  and  quinine.  Strychnia  may  be  given  in  all  cases,  but 
it  should  be  commenced  only  three  or  four  weeks  after  the  disease  haa 
become  stationary.  Although  clear  proof  of  its  utiUty  is  not  forth- 
coming, and  cannot  indeed  be  expected  in  a  disease  in  which  merely 
damaged  nerve-elements  always  tend  to  recover,  the  drug  has  a^ 
definite  influence  on  the  nutrition  of  the  structures  that  are  specially 
affected,  and  it  is  reasonable  to  suppose  that  it  promotes  their 
recovery,  and  is  capable  of  rendering  this  greater.  But  an  agent  that 
has  so  ]>owerful  an  influence  on  function  (and  therefore  on  nutrition) 
is  likely  to  do  harm,  rather  than  good,  until  normal  conditions  are 


ACUTE  ATROPHIC  PARALYSIS.  421 

being  restored.  If  functioii  oannot  respond  properly  to  a  stimulus,  it 
is  not  likely  that  nutrition  can  be  influenced  aright.  Hence,  the  more 
severe  the  lesion  the  longer  time  should  elapse  before  strychnia  is 
commenced,  and  the  smaller  the  dose  that  should  at  first  be  given. 
It  is  probably  never  either  necessary  or  desirable  to  give  it  by  hypo- 
dermic injection  in  this  disease. 

The  disease  frequently  occurs  at  the  age  at  which  any  illness  causes 
the  hindrance  to  general  development  that  constitutes  the  condition 
known  as  rickets,  and  this  fact  should  be  remembered  in  treatment. 
In  the  case  of  children  who  are  still  in  the  period  of  the  first  denti- 
tion, it  is,  therefore,  wise  to  anticipate  the  danger  by  giving,  during 
convalescence,  those  agents  that  have  most  influence  in  counteracting 
the  tendency,  especially  cod-liver  oil  and  iron ;  the  lacto-j^hosphate  of 
lime  and  iron  is  especially  useful. 

The  use  of  electricity  is  an  important  part  of  the  treatment,  although 
for  reasons  very  different  from  those  that  first  led  to  its  use,  and  it  is 
desirable  that  they  should  be  clearly  comprehended  to  secure  its 
Tjenefit  and  prevent  its  harmful  or  useless  employment.  There  is  no 
evidence  that  it  can  or  does  influence  the  process  of  recovery  of  the 
damaged  elements  in  either  the  spinal  cord  or  the  nerves.  Moreover, 
as  long  as  the  nutrition  of  these  is  actively  disordered,  its  stimulating 
influence  is  likely  to  increase  the  derangement  rather  than  promote 
the  restoration  of  a  normal  state.  The  reasons  for  its  use  depend  on 
the  fact  that  the  disease  entails  nerve-degeneration,  and  are  essen- 
tially the  same  as  those  that  justify  and  determine  its  employment  in 
other  nerve  lesions,  and  have  been  already  explained.  The  muscular 
fibres  whose  nerves  are  degenerated  suffer  changes  in  nutrition, 
and  ultimately  perish  if  no  nerve-regeneration  occurs.  While  the 
influence  normally  exerted  by  and  through  the  nerve-fibres  is  in 
abeyance,  the  muscles  are  destitute  of  this  influence,  and  without  any 
functional  stimulation.  If  they  are  excited  from  time  to  time  by 
electricity,  their  sensitiveness  to  stimulation  is  distinctly  increased, 
and  this  not  only  to  electricity,  but  to  the  voluntary  stimulus.  This  is 
clearly  shown  by  cases  that  have  been  untreated ;  the  muscles  may 
not  respond  at  aU  to  the  first  application,  but  when  they  have  been 
galvanised  two  or  three  times  a  distinct  contraction  may  be  obtained, 
and,  within  a  week,  some  voluntary  power  may  return.  Wherever 
cell  and  fibre  have  perished,  nothing  that  electricity  effects  can 
be  of  service  ;  but  where  there  has  been  damage,  not  destruction,  and 
the  nerve-fibres  slowly  recover,  but  regain  their  influence  on  nutrition, 
and  their  capacity  for  conveying  impulses,  only  after  some  months, 
the  failure  of  muscular  nutrition  may  be  disproportionately  great,  and 
may  even  render  useless  some  regained  nerve-power.  This  result 
electricity  is  probably  able  to  prevent.  It  does  not,  as  far  as  we 
can  perceive,  prevent  or  even  lessen  the  visible  wasting  of  the 
muscles  ;  it  is  powerless  to  counteract  the  effects  of  a  destructive 
lesion  ;  but  it  does  seem  to  hasten  recovery  from  the  effects  of  partial 


422  SPINAL   COED. 

damage,  and  to  lessen  fhe  nltimate  degree  of  this  damage.  It  does  so 
solely  by  its  influence  on  the  muscular  tissue,  and  only  voltaic  elec- 
tricity can  stimulate  this  tissue  when  the  nerve-fibres  are  degenerated. 
The  mode  of  application  should  be  the  same  as  for  nerve  lesions. 
The  muscle  is  only  stimulated  when  the  circuit  is  interrupted,  as  by 
repeatedly  stroking  the  muscles  with  one  terminal  (a  sponge,  or  con- 
ductor covered  with  water-holding  material,  well  wetted  with  salt  and 
water),  which  should  be  lifted  from  the  skin  between  each  stroke. 
The  other  terminal  is  kept  still ;  it  may  be  placed  on  the  upper  part 
of  the  muscle,  where  its  nerve  enters  it.  By  some  this  terminal  is 
placed  on  the  spine  over  the  affected  region  (and  then  a  larger  flat 
terminal  is  employed),  under  the  impression  that  the  electricity  may 
influence  the  morbid  process  in  the  spinal  cord;  but  there  is  na 
evidence  even  that  the  spinal  cord  is  reached  by  electricity  so  applied. 
When  applied  to  the  muscles,  the  strokes  may  be  made  with  the 
negative  terminal,  since  each  pole  will  influence  the  tissue,  and  the 
normally  greater  irritability  to  the  negative  often  persists.  The  appli- 
cation can  be  made  perfectly  well  by  an  intelligent  nurse,  since  the 
diffusion  of  the  voltaic  current  is  so  great  that  it  is  sure  to  reach  the 
affected  muscles.  Each  time  the  sponge  is  placed  on  the  skin  the 
affected  muscles  should  be  seen  to  move  or  swell  up  in  slight  contrac- 
tion, and  it  is  desirable  to  use  as  many  cells  of  the  battery  as  wiU 
produce  this  result.  Sometimes  this  cannot  be  done  without  causing 
so  much  pain  as  to  distress  the  child,  but  in  many  instances  the 
distress  is  really  due  to  a  needlessly  strong  and  painful  current 
having  at  some  time  been  used,  and  an  amount  of  alarm  produced 
that  the  child  never  gets  over.  It  is  on  this  account  that  it  is  se 
important  to  make  a  test  examination  with  great  care,  and  to  employ 
the  less  painful  faradic  shock.  Emotional  disturbance  may  generally 
be  avoided  by  commencing  with  a  very  weak  current.  It  is  better, 
indeed,  to  commence  with  no  current  at  all,  applying  the  sponges  in 
the  way  directed,  so  that  the  child  may  be  familiar  with  them, 
and  cease  to  fear  them.  Then  two  or  three  cells  may  be  used,  and  the 
strength  gradually  increased  day  by  day.  In  this  way  a  current  strong^ 
enough  to  cause  contraction  will  often  be  tolerated.  If,  however, 
in  spite  of  these  precautions,  this  strength  cannot  be  employed 
without  distressing  and  disturbing  the  child,  the  attempt  to  obtain 
muscular  contraction  should  not  be  persevered  in,  but  only  such 
a  cui'rent  employed  as  does  not  disturb  the  child.  An  influence  on 
nutrition,  if  slighter,  is  still  exerted,  and  if  the  application  is  con- 
tinued for  a  longer  time  it  is  probable  that  equal  good  is  done.  The 
electrical  treatment  may  be  commenced  at  the  end  of  the  fourth  week 
after  the  onset.  It  should  not  be  used  earlier,  lest  it  excite  increased 
disturbance  in  the  spinal  cord.  The  application  need  only  be  made  to 
those  muscles  in  which  faradic  irritabihty  is  lowered  or  lost.  Other  mus- 
cles will  recover  without  its  aid,  or  will  be  uninfluenced  by  electricity. 
Another  measure  which  should  be  employed  is  systematic  rubbing 


ACUTE  ATROPHIC  PARALYSIS.  423 

of  tie  limbs.  This  stimulates  the  circulation,  which  is  always  defec- 
tive, as  the  blue,  cold  surface  shows.  It  no  doubt  also  increases  the 
movement  of  the  fluids  in  the  tissues  outside  the  vessels,  and  so 
probably  increases  the  interchange  of  material,  and  promotes  nutri- 
tion. The  muscles  should  be  daily  rubbed  and  gently  Imeaded, 
upward  rubbing  being  especially  useful.  No  liniment  is  required ;  the 
uncovered  hand  answers  best.  Cod-liver  oil  is  sometimes  rubbed  on ; 
a  minute  quantity  is  probably  rubbed  in,  without  good  or  harm. 
Great  care  should  be  taken  at  all  times  to  keep  the  affected  limbs  as 
warm  as  possible. 

In  all  cases  in  which  the  trunk  muscles  are  weak,  even  in  slight 
degree,  bronchial  catarrh  must  be  guarded  against  with  great  care. 
In  such  cases  the  muscles  of  respiration  are  weakened,  and  although 
normal  breathing  may  not  be  impaired,  the  diminution  in  strength 
may  render  an  acute  bronchial  catarrh  rapidly  fatal,  and  this  even, 
months  after  the  onset  of  the  paralysis. 

When  some  voluntary  power  is  regained  the  systematic  use  of  the 
muscles  is  of  great  service,  and  shoidd  be  carefully  attended  to. 
Systematic  j)lanned  movements  may  generally  be  adopted,  and  an 
improvement  of  muscular  nutrition  and  power  may  be  secured  much 
earlier  than  if  the  limbs  are  left  to  the  chance  influence  of  such  move- 
ments as  a  child  spontaneously  adopts.  Most  of  the  good  that  is 
done  by  "movement  cures"  may  be  obtained  by  such  simple  mus- 
cular exercises  as  can  be  readily  arranged  with  the  help  of  a  little 
consideration  on  the  part  of  the  doctor,  and  can  be  efficiently  carried 
on  at  home,  given  the  necessary  perseverance  on  the  part  of  those 
who  have  charge  of  the  patient,  and  which,  unfortunately,  is  less 
often  forthcoming.  Such  movements  need  to  be  specially  arranged 
to  meet  the  exigencies  of  each  case,  so  as  to  call  into  action  those 
muscles  that  are  weak  but  not  powerless,  or  that  may  supplement 
the  muscles  that  are  paralysed. 

A  very  important  element  in  the  management  of  the  chronic  stage 
is  the  prevention  and  treatment  of  the  muscular  contractions,  and  of 
the  deformities  to  which  these  give  rise.  The  contractions  cannot  be 
entirely  hindered,  but  they  may  often  be  prevented  reaching  a  high 
degree  by  careful  attention  to  the  position  of  the  lim.bs,  and  by 
watching  for  and  treating  the  earliest  indication  of  shortening  of  the 
muscles.  It  is  needless  to  discuss  in  detail  the  varied  influence  of 
posture  in  these  cases  ;  careful  and  frequent  examination  of  the  patient 
will  show  whether  any  deviation  from  normal  relations  is  being  deve- 
loped, and  its  counteraction  is  chiefly  a  matter  of  common  sense  and 
a  little  practical  ingenuity.  Of  especial  importance,  however,  are  the 
prevention  of  the  curvatures  of  the  spine  which  are  caused  by  allowing 
the  patient  to  sit  up  before  the  back  muscles  have  regained  the  neces- 
sary power,  and  the  prevention  of  the  shortening  of  the  flexors  of  the 
hip  and  knee  joints  that  occurs  when  the  patient  is  allowed  to  lie  in 
bed  with  the  legs  drawn  up.     More  difficult  of  prevention  is  the  con- 


424  SPINAL  CORD. 

traction  of  the  calf  muscles,  often  aggravated  in  consequence  of  the 
lessened  growth  of  the  limb.  Something  may  be  done  during  the 
process  of  rubbing  for  the  prevention  of  deformities.  While  the  con- 
tracted muscles  are  rubbed  upwards  they  should  be  extended  gently 
but  firmly.  Thus,  if  the  caK  muscles  are  shortened,  the  foot  should  be 
steadily  pressed  upwards  while  the  calf  is  rubbed.  A  slipper  with 
elastic  straps  to  a  knee-band  may  be  worn  at  night,  often  with  great 
benefit.  For  developed  deformities,  due  to  great  shortening  of  the 
muscles,  surgical  treatment  by  splints  or  tenotomy  is  usually  necessary, 
and  is  often  important  because  it  enables  the  patient  to  use  the  limb 
in  ways  that  would  be  impossible  without  it. 

For  this  and  other  reasons  mechanical  appliances  are  of  unques- 
tionable value.  Counteracting  deformities  and  supplementing  weak 
muscles,  they  often  enable  a  child  to  walk  who  could  not  do  so 
without  their  aid,  and  power  is  often  increased  to  a  rejnarkable  extent 
in  indirect  ways.  In  all  these  cases,  however,  the  instruments  require 
frequent  attention  and  alteration  to  adapt  them  to  the  changes  due  to 
growth,  and  patience  is  required  in  the  management  of  these  cases 
through  their  long  and  tedious  course. 

Two  facts  deserve  a  final  emphasis.  At  the  end  of  six  months  all 
possible  lecDvery  is  nearly  completed,  and  certainly  at  the  end  of  a 
year  the  lesion  has  become  a  cicatrix,  and  further  improvement  will  be 
merely  by  the  slow  growth  of  the  muscle  that  has  recovered,  under 
the  stimulus  of  use.  Secondly,  this  process  of  slow  improvement  as 
the  result  of  use  will  go  on  for  years, — will  go  on  whatever  treatment 
is  adopted,  and  whether  special  measures  are  employed  or  not.  The 
therapeutic  specialist  who  carries  on  his  treatment  month  after  month 
will  claim  it  as  his  achievement,  but  it  would  occur  equally  without 
his  aid.  From  its  nature,  however,  as  already  stated,  it  may  probably 
be  always  augmented  by  some  contrivance  to  increase  the  influences 
that  are  really  improving  the  power  of  the  muscles. 

Subacute  and  Chronic  Ateophic  Spinal  Paralysis 
(subaotite  and  cheonio  polio-myelitis). 

Under  this  designation  cases  have  been  described  of  a  miscellaneous 
character,  in  which  paralysis,  followed  by  muscular  atrophy,  comes  on 
less  rapidly  than  in  the  acute  form,  its  development  occupying  from 
ten  to  thirty  days  in  the  cases  which  are  termed  "  subacute,"  and 
from  one  to  six  months  or  even  more  in  those  which  are  called 
*•  chronic."  Many  cases  included  under  this  name  differ  much  in 
their  characters,  and  are  sometimes  described  as  **  chronic  myelitis ;  " 
but  among  the  cases  placed,  even  untU  lately,  in  this  group,  are  forms 
of  multiple  neuritis.  Most  subacute  atrophic  palsies  are  due  to  nerve 
disease  ;  almost  all,  indeed,  which  present  symmetrical  palsy. 

The  cases  of  spinal  atrophic  paralysis,  in  which  the  onset  is  not 
acute,  may  be  placed  in  four  classes. 


ACUTE  ATROPHIC  PARALYSIS.  425 

(1)  Cases  of  subacute  polio-myelitis  wliicli  differ  from  tlie  acute 
form,  already  described,  only  in  their  less  rapid  development.  They 
present  the  same  initial  general  disturbance  and  wide  distribution, 
and  the  same  recovery,  except  in  a  limited  region  in  which  muscular 
wasting  occurs.  They  are  thus  distinguished  from  polyneuritis,  and 
also  by  the  irregular  distribution  of  the  symptoms.  Most  of  the  suf- 
ferers have  been  adults.  Little  is  known  of  the  exciting  cause  of  this 
form  further  than  that  it  certainly  sometimes  follows  exposure  to  cold, 
and  is  probably  sometimes  the  result  of  a  toxsemic  influence.  The 
symptoms  and  general  history  of  these  cases  present  no  important 
difference  from  the  acute  form. 

(2)  Cases  are  met  with  that  differ  from  the  type  just  described  in 
the  fact  that  their  course  is  progressive.  The  onset  is  subacute  or  sub- 
chronic,  occupying  from  a  fortnight  to  several  months,  but  instead  of 
arrest  followed  by  improvement,  the  more  or  less  rapid  onset  is  fol- 
lowed by  slower  gradual  increase,  until  at  last  wide-spread  chronic 
muscular  atrophy  is  developed.  Many  of  these  are  really  cases  of 
progressive  muscular  atrophy  with  a  subacute  onset.  For  example,  I 
have  more  than  once  known  a  case  of  this  disease,  ultimately  typical, 
to  begin  by  a  subacute  atrophic  paralysis  of  the  extensors  of  the 
wrists.  These  cases  will  be  considered  in  the  account  of  that  disease. 
Others  present  differences  from  that  malady  and  symptoms  of  more 
irregular  character,  which  show  that  the  lesions  are  distributed 
through  various  structures  in  the  cord,  and  these  must  be  regarded 
as  cases  of  chronic  myelitis  involving  the  anterior  cornua.  This  form 
also  is  chiefly  confined  to  adults.  It  occasionally  results  from  cold, 
and  sometimes  from  injury,  as  a  fall  on  the  back.  Intemperance  and 
venereal  excesses  are  also  said  to  cause  it.  The  wide-spread  muscular 
atrophy  which  sometimes  results  from  lead-poisoning  is  probably  of 
this  nature.  The  muscles  at  first  present  the  degenerative  reaction, 
or  normal  faradic  irritability  with  increase  to  volfcaism;  sometimes, 
however,  there  is  a  loss  to  both  f aradism  and  voltaism.  The  sphincters 
usually  escape.  In  many  cases  the  affection  exhibits  a  persistently 
progressive  character;  muscular  atrophy  slowly  increases  and  ex- 
tends, until  the  case  ultimately  resembles  one  of  progressive  muscular 
atrophy,  and  the  patient  dies  at  the  end  of  one  or  two  years  from 
exhaustion,  or  from  interference  with  the  respiratory  movements.  In 
other  cases  the  atrophy,  after  slowly  progressing  for  many  months, 
becomes  stationary,  and  considerable  improvement  may  ultimately 
take  place.  This  course  is  seen  especially  in  traumatic  cases.  Few- 
observations  on  the  pathological  anatomy  of  the  affection  have  been 
made.  Cornil  and  Lepine  found,  in  one  case,  at  the  end  of  four 
years,  softening  of  the  lower  part  of  the  spinal  cord,  chiefly  in  the 
anterior  cornua,  disappearance  of  the  ganglion-cells,  increase  of  the 
connective  tissue,  and  sclerosis  of  the  white  substance  around  the 
anterior  horns. 

The  diagnosis  from  the  first  form  rests  on  the  slow  onset  and  pro- 


426  SPINAL  COED. 

gressive  course  of  tlie  disease.  Althougli  the  degenerative  electrical 
reaction  may  be  found,  it  is  frequently  absent,  and  this  is  another 
distinction  from  the  acute  variety.  On  the  other  hand,  the  fact  that 
paralysis  precedes  wasting  is  a  distinction  from  progressive  muscular 
atrophy.  In  the  most  chronic  form,  however,  this  distinction  fails, 
and  cases  are  met  with  which  present  a  gradation  between  the  two 
diseases. 

The  prognosis  is  grave  except  in  traumatic  cases,  but  is  influenced 
by  the  observed  rate  of  progress,  and  the  presence  or  absence  of  any 
indications  of  arrest.  When  the  result  of  injury,  considerable  im- 
provement is  not  uncommon,  and  it  is  remarkable  how  great  a  degree 
of  paralysis  and  atrophy  may,  in  these  cases,  ultimately  pass  away 
almost  completely.  The  treatment  of  the  disease  must  be  influenced 
by  the  rapidity  of  its  onset.  In  the  subacute  stage  and  form  it  should 
be  conducted  on  the  same  principles  as  that  of  acute  cornual  myelitis. 
In  the  chronic  form  the  treatment  must  be  the  same  as  for  progressive 
muscula-r  atrophy. 

(3)  Many  subacute  and  chronic  cases,  which  have  been  described 
as  atrophic  spinal  paralysis,  are  peripheral,  not  central  in  their  nature, 
and  are  cases  of  multiple  neuritis,  the  symptoms  and  diagnostic 
indications  of  which  have  been  already  described.  The  history  of  the 
subacute  spinal  disease  has  been  largely  written  from  cases  of  multiple 
neuritis.  In  the  statements  just  made  this  fact  has  been  kept  in 
view.  While  multiple  neuritis  may  simulate  closely  chronic  polio- 
myelitis, it  is  certain  that  the  converse  is  also  true,  and  the  central 
affection  has  been  sometimes  thought  to  be  peripheral.  The  facts  at 
present  available  suggest  that  the  central  and  peripheral  structures  of 
common  function  possess  common  susceptibilities  to  the  action  of 
morbid  influences,  and  there  is  still  danger  that  the  two  classes  may 
be  confused.  The  most  important  criterion  is  the  greater  irregularity 
in  distribution  of  the  central  disease, — the  more  perfect  symmetry  of 
the  peripheral  affection.  The  presence  of  tenderness  of  the  nerve- 
trunks,  and  of  the  structures  to  which  the  nerves  are  distributed,  is  of 
more  value,  when  it  exists,  than  is  the  suggestion  afforded  by  its 
absence  that  the  disease  is  central,  because,  in  the  periphery,  only 
fibres  of  one  function  may  suffer :  we  may  then  have  none  of  the 
symptoms  that  we  commonly  associate  with  neuritis,  and  look  for  as 
proof  of  its  existence.  In  pui'ely  motor  neuritis  there  may  be  no 
tenderness  or  pain.  But  such  an  affection  is  either  unilateral  or 
perfectly  symmetrical.  Imperfect  symmetry  indicates  an  affection  of 
the  cord. 

(4)  A  family  form  of  slow  muscular  atrophy  in  children  with  changes 
in  the  anterior  horn  ceUs  has  been  described  in  the  last  few  yeais  by 
Hoffmann  and  others.  It  will  be  referred  to  when  we  come  to  speak 
c  f  the  myopathic  atrophies  with  which  it  has  some  affinities. 


ACUTE    ASCENDING  PARALYSIS.  427 


ACUTE    ASCENDING    PARALYSIS. 

Ascending  paralysis,  which  commences  in  the  legs  and  ascends  to 
the  muscles  of  the  trunk,  the  arms,  muscles  of  the  neck,  the  diaphragm 
and  the  pharynx,  is  a  feature  of  several  diseases — meningeal  hsenior- 
rhage,  ascending  myelitis,  &c. ;  but  it  occurs  also  in  cases  in  which  the 
spinal  cord,  after  death,  appears  healthy  to  both  naked-eye  and 
microscopical  examination.  Such  cases  have,  however,  certain  charac- 
teristic features  which  make  it  desirable  to  distinguish  the  condition 
from  other  diseases,  and  the  affection  has,  therefore,  been  provisionally 
termed  ''acute  ascending  paralysis,"  in  the  absence  of  any  evidence  of 
its  nature.  It  was  first  described  by  Landry  in  1859,  and  hence  is 
often  called  Landry's  paralysis.  Acute  ascending  paralysis  is  a  most 
formidable  malady,  most  cases  proving  fatal  in  a  few  days.  Its 
nature  is  mysterious,  but  recent  discoveries  regarding  midtiple 
neuritis  have  disclosed  instructive  analogies  between  the  two  affec- 
tions. These  have,  indeed,  led  some  observers  to  the  opinion  that  this 
ascending  paralysis,  without  organic  central  lesion,  is  an  affection  of 
the  nerves.*  As  we  shall  see,  it  is  not  probable  that  this  opinion  is 
correct,  although  it  is  possible  that  the  nerves,  as  well  as  the  centres, 
are  sometimes  affected. 

Causes. — The  etiology  of  the  disease  resembles,  in  general,  that  of 
acute  multiple  neuritis  more  nearly  than  that  of  any  other  affection. 
The  disease  affects  males  more  frequently  than  females.  It  occurs 
chiefly  between  twenty  and  forty  years  of  age,  but  has  been  observed 
in  rare  cases  in  older  and  younger  persons,  and  even  in  children. 
Some  sufferers  have  been  the  subjects  of  alcoholism.  Severe  exposure 
to  cold  has  been  the  apparent  cause  in  many  instances.  In  other  cases 
the  disease  has  occurred  under  conditions  such  as  cause  toxsemic 
states,  or  after  the  occurrence  of  some  known  blood  disease.  Thus 
it  has  occurred  during  convalescence  from  some  general  disease, 
smallpox,  diphtheria,  typhoid  fever.  It  has  also  followed  influenza 
and  febrile  diseases  of  obscure  nature.  In  these  cases,  it  should  be 
noted,  the  disease  has  followed  at  an  interval  of  a  week  or  a  few  weeks. 
It  also  soBietiiiies  succeeds  some  traumatic  process,  such  as  a  wound, 
and  here  also  after  an  interval,  and  usually  when  the  wound  was 
apparently  healed.  I  have  known  it  thus  to  follow  an  attack  of  pelvic 
cellulitis.  In  these  respects  it  closely  resembles  polyneuritis ;  and  it 
has  been  observed,  in  severe  form,  in  the  subjects  of  old  alcoholic 
neuritis.  The  disease  has  been  repeatedly  observed  in  the  subjects  of 
syphilis,  and  has  apparently  been  arrested  by  the  treatment  for  this 
disease,  t     The  circumstance  that  when  some  malady  or  woimd  pre- 

*  See  Eoss  and  Judson  Bury,  'Treatise  on  Peripheral  Neuritis.' 
t  Much  weight  should  not  be  placed  on  this  fact  as  proof  of  causal  relationship, 
because  (apart  from  the  fallacy  of  independent  cessation)  it  is  probable  that  the 
treatment  that  is  effective  against  the  virus  of   syphilis  is  also  effective  against 
other  blood-states  that  depend  on  agents  of  similar  nature. 


428  SPINAL    COED. 

ceded  tte  disease,  tlie  former  was  often  of  a  trivial  character,  prevents 
surprise  at  the  fact  that,  in  many  cases,  no  influence  could  be  traced 
to  which  the  disease  could  be  ascribed. 

Symptoms. — Premonitory  symptoms  have  been  noted  in  some  cases 
■ — general  malaise,  pains  in  the  head  and  back,  tingling  in  the  extremi- 
ties— for  a  few  days  or  a  week  before  the  onset.  The  first  definite 
symptom  is  usually  weakness  of  the  legs,  often  commencing  in  one 
and  spreading  to  the  other.  The  weakness  increases  rapidly,  so  that 
the  power  of  standing  is  lost,  sometimes  in  a  few  hoTirs,  sometimes  at 
the  end  of  two  or  three  days  ;  it  goes  on  to  complete  paralysis,  with 
relaxation  of  the  muscles.  As  the  legs  become  motionless  the  muscles 
of  the  trunk  become  weak,  first  of  the  pelvis,  loins,  and  abdomen 
then  of  the  thorax.  The  weakness  next  invades  the  arms  ;  either  the 
upper  arm  muscles  or  those  of  the  forearm  and  hand  may  be  first 
attacked,  and  one  arm  is  often  weakened  before  the  other.  The 
paralysis  of  the  arms  may  become  absolute,  like  that  of  the  legs,  or 
some  power  of  movement  may  remain.  The  diaphragm  and  neck 
muscles  then  suffer,  and  difficulty  of  swallowing  comes  on,  sometimes 
wilh  paralysis  of  the  muscles  of  the  palate,  and  often  speech  becomes 
difficult,  nasal,  and  indistinct.  The  inability  to  swallow  may  become 
so  great  that  the  patient  has  to  be  fed  through  a  tube,  and  the  para- 
lysis may  involve  the  muscles  of  articulation  to  such  an  extent  that 
utterance  may  be  altogether  unintelligible.  There  is  often  dyspnoea 
from  the  weakness  of  the  muscles  of  respiration,  or  from  interference 
with  the  respiratory  centre  in  the  medulla,  and  the  cardiac  centre  may 
also  be  involved.  The  affection  of  the  bulbar  nerves  is  determined  by 
functional  relations ;  thus  the  lips  may  be  paralysed  with  other 
muscles  of  articulation  when  the  upper  part  of  the  face  is  unaffected. 
In  rare  cases  the  eye  muscles  are  paralysed  in  some  degree,  usually 
manifested  only  as  loss  of  accommodation,  inequality  or  dilatation  of 
the  pupils,  impaired  reaction  to  light,  or  slight  strabismus. 

The  tinghng  and  analogous  subjective  sensory  disturbance  has  been 
followed,  in  some  cases,  by  hyperaesthesia  of  the  skin  and  tenderness 
of  the  muscles,  but  it  is  probable  that  in  such  cases  there  has  been 
multiple  neuritis.  There  may  be  some  blunting  of  sensibility  in  the 
extremities,  but  there  is  definite  loss  of  sensation  only  in  some 
severe  cases  of  the  typical  disease.  A  firm  touch  can  usually  be  per- 
ceived anywhere ;  perception  of  painful  impressions  and  of  heat  or 
cold  is  sometimes  delayed.  At  first,  reflex  action  is  lost  in  the  affected 
limbs,  both  cutaneous  reflex  action  and  myotatic  irritability.  In  cases 
rapidly  fatal  the  loss  has  continued  tiU  death.  In  cases  that  have 
recovered  reflex  action  has  returned,  but  recorded  cases  have  presented 
considerable  differences  in  this  respect,  and,  as  there  is  some  doubt  as 
to  the  nature  of  many  non-fatal  cases,  there  is  some  uncertainty  as  to 
this  point.  In  some  the  myotatic  irritability  has  soon  returned,  and 
has  even  become  excessive.      In  the  majority  it  has  remained  absent. 


ACUTE    ASCENDING    PAEALYSIS.  429 

and  not  until  all  paralytic  symptoms  have  disappeared  has  the  knee- 
jerk  returned. 

In  spite  of  the  early  flaccidity  of  the  muscles,  if  life  is  prolonged 
they  rarely  present  either  wasting  or  change  in  electrical  irritabihty. 
A  trifling  reduction  in  size  may  occur,  but  there  is  no  muscular  atrophy 
such  as  occurs  in  polio-myelitis,  and  even  after  several  weeks  the  most 
careful  examination  fails  to  reveal  any  abnormal  electrical  reaction — 
a  very  important  feature  of  the  disease.  Exceptional  cases,  which  do 
present  the  degenerative  reaction  in  the  muscles,  seem  more  closely 
allied  to  a  universal  polio-myelitis,  from  which,  indeed,  this  disease 
cannot  be  sharply  separated.  The  sphincters,  moreover,  escape  in  the 
vast  majority  of  cases,  but  not  in  all.  There  is  no  tendency  to  the 
occurrence  of  bedsores.  The  cerebral  fiinctions  are  not  usually 
involved,  and  the  state  of  the  patient  who,  with  unimpaired  intellect, 
cannot  express  himself  either  by  speech  or  gesture,  is  painful  in  the 
extreme.  In  exceptional  cases,  however,  there  is  mental  dulness  or 
slight  delirium,  as  if  from  the  influence  of  a  blood- state  on  the  brain. 
In  girls  this  may  take  the  form  of  hysterical  manifestations,  by  which 
the  nature  of  the  disease  may  be  masked. 

As  a  rule  there  is  no  elevation  of  temperature,  even  during  the  rapid 
development  of  the  symptoms,  but  in  one  or  two  cases,  which  were  not 
fatal,  brief  pyrexia  attended  the  onset,  and  in  a  few  others  moderate 
febrile  disturbance  (2°  or  3°)  occurred  at  a  late  period.  Profuse 
sweating  has  been  occasionally  noted.  Enlargement  of  the  spleen 
(first  noted  by  Westphal),  such  as  occurs  in  other  acute  diseases,  ia 
very  frequent. 

Thus  the  chief  feature  of  the  disease  is  an  almost  purely  motor 
paralysis,  progressive  in  character,  with  relaxation  of  the  muscles 
during  the  acute  stage  of  the  disease.  It  usually  has  an  ascending 
course,  but  irregular  cases  are  met  with  in  which  the  order  of  invasion 
is  varied.  Thus  the  arms  are  occasionally  involved  before  the  legs, 
the  diaphragm  before  the  intercostals ;  the  bulbar  symptoms  have 
been  known  to  precede  the  others,  and  I  have  seen  them  reach  a  high 
degree  with  a  fatal  involvement  of  the  cardiac  centre  before  the  upper 
muscles  of  respiration  were  affected,  or  the  hands  quite  powerless. 
Analogous  cases  have  been  recorded  by  others,  the  special  features 
and  course  being  those  characteristic  of  the  disease.  In  some  cases, 
however,  of  irregular  course,  changes  have  been  found  in  the  electric 
irritability  of  the  muscles ;  it  is  probable  that,  in  such  cases,  the  peri- 
pheral nerves  are  affected,  although  we  cannot,  on  this  account,  at 
pi-esent  separate  these  from  the  typical  cases  of  the  disorder — a  point 
that  will  be  explained  in  connection  with  its  pathology. 

The  disease  varies  considerably  in  the  rate  of  its  progress.  It  may 
run  its  course  and  end  fatally  in  forty-eight  hours.  Death  usually 
results  from  either  respiratory  or  cardiac  paralysis — the  latter  in  the 
cases  of  irregular  course.  A  large  proportion  of  the  fatal  cases  last 
less  than  a  week.     On  the  other  hand,  the  disease  may  only  attain  its 


430  SPINAL   COED. 

lieiglit  at  the  end  of  two,  three,  or  even  four  weeks.  In  some  cases, 
apparently  of  the  same  character,  the  paralysis  of  the  limbs  has  not 
been  complete.  There  may  be  a  rapid  extension  of  the  paralysis  to  a 
certain  point,  and  it  may  then  cease  to  spread,  and  the  limbs  last  and 
least  affected  may  slowly  regain  power.  A  patient  may  lie  for  days 
in  a  condition  of  universal  palsy,  save  for  a  little  respiratory  power, 
and  then  improvement  begin.  As  a  rule,  the  order  of  recovery  of  the 
muscles  is  the  opposite  to  that  of  their  invasion.  In  favourable  cases 
the  recovery  of  power  is  usually  slow;  two  or  three  months  often 
elapse  before  the  weakness  entirely  disappears.  Occasionally  there 
has  been  a  more  rapid  improvement,  and  the  patient  has  been  well  in  a 
few  weeks. 

Pathologt. — The  most  careful  and  skilled  examination  has  often 
failed  to  discover  any  morbid  appearance  in  the  spinal  cord,  nerves,  or 
muscles.  Minute  hsemorrhages,  met  with  occasionally,  have  probably 
occurred  during  the  last  moments  of  life.  In  other  cases,  apparently 
similar  to  the  others,  vascular  engoragement  has  been  found  in  the  grey 
matter  of  the  cord,  the  significance  of  which  is  increased  by  leucocytal 
accumulations  around  the  vessel.  Changes  in  the  ganglion-cells  in 
the  more  acute  cases  have  also  been  found,  swelling  and  blurred 
outline.*  Of  late  attention  has  been  paid  to  the  peripheral  nerves 
and  sHght  indications  of  acute  changes,  both  parenchymatous  and 
interstitial,t  have  been  found  in  them,  but  only  in  exceptional  cases, 
and  peripheral  neuritis  seems  to  play  no  part  in  the  typical  disease, 
although  sometimes  simulating  it.  In  addition  to  the  enlargement  of 
the  spleen  observed  during  life,  and  found  also  after  death,  the  mesen- 
teric glands  have  been  found  swollen,  and  also  the  closed  follicles  of 
the  intestines.  Organisms  have  been  found  in  the  glands,J  and 
culture  of  portions  of  the  nerve-centres  yielded,  in  one  case,  a  bacillus 
resembling,  but  not  identical  with,  that  of  splenic  fever.  §  Other 
observers  have  searched  for  them  without  success  in  all  cases  of 
typical  character. 

These  negative  facts,  taken  in  conjunction  with  the  conditions  under 
which  the  disease  occurs,  and  with  the  course  of  the  malady,  have 
suggested  the  idea  of  a  toxic  influence  acting  on  the  nerve-centres ; 
and  this  idea  receives  support  from  the  discovery  that  acute  swelling 
of  the  spleen  is  common,  and  of  the  lymphatic  glands  not  rare — 
lesions  that  indicate  a  morbid  blood-state.  The  limitation  by  func- 
tion of  many  toxsemic  palsies,  e.  g.  that  of  accommodation,  supports 
this  view,  since  the  isolated  acute  paralysis  of  functional  centres,  not 
anatomically  separate  from  others,  is  known  only  as  a  consequence  of 
a  toxic  influence.  Further  confirmation  is  afforded  by  the  fact  that 
cases  of  acute  multiple  neuritis,  certainly  due  to  a  toxaemia,  may  run  a 

*  Immermann,  Marinesco  and  Marie,  Bailey  and  Ewing, 
t  Eisenlohr,  •  Deut.  med.  Wocbenschr.,'  1890,  No.  38. 
X  Baumgaiten. 
§  Marie  and  Marinesco. 


ACUTE    ASCENDING   PARALYSIS.  431 

similar  course.  It  lias,  indeed,  been  conjectured  that  the  neryes  are 
always  the  structures  impaired,  but  this  exclusive  view  is  disproved 
by  the  normal  state  of  the  nerves  in  cases  in  which  the  grey  substance 
of  the  spinal  cord  has  been  found  to  be  changed  and  by  the  common 
absence  of  altered  excitability  of  the  muscles,  since  the  motor  nerves 
seem  never  to  escape  invariably  in  peripheral  neuritis  of  any  variety, 
however  common  their  freedom  from  affection  in  the  type.  But  the 
nerves  suffer  also  in  some  cases,  and  there  seem  to  be  gradations  to 
the  cases  of  acnte  multiple  neuritis  of  ascending  course  described  at 
p.  162.  All  the  facts  point  to  the  conclusion  that  the  disease  is  due 
to  a  toxin  which  has  a  specific  action  on  the  motor  neurons  in  the 
spinal  cord,  chiefly  on  the  anterior  gi'ey  matter,  sometimes  also 
affecting  the  motor  nerve-fibres  proceeding  from  them.  Sometimes 
it  has  a  wider  influence  on  the  spinal  cord,  and  its  effects  can  be 
traced  into  the  white  substance  or  the  posterior  horns,  possibly  as  an 
extension  of  the  inflammatory  changes  it  induced.  These  are  similar 
to  those  that  attend  any  acute  disturbance  of  function;  the  toxic 
influence  is  mysterious  in  nature  and  origin,  but  seems  to  resemble 
that  which  causes  polio-myelitis,  in  which  the  morbid  process  presents 
variations  and  may  also  involve  the  nerves.  The  toxine  may  be  pro- 
duced by  the  agency  of  bacteria,  but  the  evidence  of  this  is  incon- 
clusive ;  some  cases  have  followed  exposure  to  cold. 

DiAaNosis. — The  disease  is  recognised  by  the  rapid  development  of 
paralysis,  usually  but  not  invariably  ascending,  with  relaxation  of  the 
muscles,  and  with  loss  of  reflex  action,  without  considerable  pain  or, 
generally,  any  loss  of  sensation,  and  if  the  patient  survives,  without 
wasting  of  the  muscles  or  change  in  electrical  irritability.  The  latter 
characteristic  distinguishes  the  disease  from  acut«  atrophic  paralysis, 
while  the  absence  of  pain  in  the  back  and  of  spasm  is  a  distinction 
from  meningeal  hsemorrhage.  The  diagnosis  from  general  ascending 
myelitis  has  been  already  considered;  it  rests  especially  on  the  in- 
volvement of  all  the  functions  of  the  cord  in  inflammation.  The 
distinction  from  multiple  neuritis  has  been  mentioned  in  the  account 
of  that  disease  (p.  174),  but,  it  may  be,  is  not  always  absolute,  since 
the  nerves  probably  suffer,  as  well  as  the  cord,  in  some  cases  of 
ascending  paralysis.  Further,  the  cases  of  multiple  neuritis  simulating 
this  affection,  the  nature  of  which  has  been  demonstrated  pathologi- 
cally, have  usually  run  a  more  prolonged  course,  several  weeks  or  even 
months.  The  distinction  from  polyneuritis,  founded  on  the  course  of 
the  ascending  form,  is  subject,  however,  to  the  reservation  that  while 
ascension  to  the  arm  through  the  trunk  is  a  feature  of  the  central 
disease,  this  is  not  excluded  by  a  more  irregular  course,  and  the 
character  of  the  individual  symptoms  must  be  taken  into  consideration 
(see  also  p.  429). 

Pkoqnosis. — The  affection  is  one  of  extreme  gravity.  The  danger 
to  life  is  in.  proportion  to  the  interference  with  respiration  and  with 
the  functions  of  the  medulla  oblongata,  especially  with  the  cardiac 


432  SPINAL    CORD. 

centre,  and  also  to  tlie  rapidity  with  TvTiicli  tlie  palsy  comes  on.  But 
the  cases  which  develop  with  comparative  slowness  are  not  devoid  of 
danger.  Even  when  the  symptoms  only  reach  their  height  at  the  end 
of  three  or  four  weeks,  death  may  occur  in  the  same  way  as  in  the 
more  rapid  cases.  On  the  other  hand,  recovery  has  been  known 
although  the  patient  has  lost  all  power  of  motion  at  the  end  of  the 
second  day.  The  danger  is  great  as  long  as  the  symptoms  are  in- 
creasing, and  only  when  distinct  improvement  can  he  recognised  is  it 
justifialble  to  anticipate  recovery.  The  earlier  the  bulbar  symptoms 
appear,  the  more  serious  is  their  significance.  Mental  symptoms  also 
increase  the  gravity  of  the  prognosis,  as  they  usually  indicate  a  severe 
blood-change,  the  effects  of  which  on  the  nervous  system  are  likely  to 
reach  a  high  degree. 

Treatment. — During  the  early  stage  of  an  attack  of  acute  ascend- 
ing paralysis,  in  the  absence  of  other  indications,  the  treatment  should 
be  that  suitable  for  myelitis,  since,  at  the  onset,  the  diagnosis  between 
the  two  diseases  can  never  be  certain.  A  warm  bath,  or  still  better, 
a  vapour  bath,  should  be  given  if  the  symptoms  followed  exposure  to 
cold.  It  should  be  followed  by  counter-irritation  over  the  spine  by  a 
long,  narrow  mustard  plaster.  More  energetic  counter-irritation,  even 
the  actual  cautery,  has  been  recommended.  The  body  should  be  kept 
in  as  perfect  rest  as  possible,  and  on  the  side  rather  than  on  the  back. 
In  very  few  cases  have  drugs  appeared  to  exert  any  influence  on  the 
course  of  the  disease,  and  the  malady  is  so  rare  that  experience  accu- 
mulates slowly.  Salicylate  of  soda  seems  to  deserve  a  trial  in  cases 
that  follow  exposure  to  cold.  Ergotin  has  been  given,  and  one  case  in 
which  it  was  used  deserves  special  mention.  The  patient  was  a  man 
aged  fifty-seven,  who,  a  week  after  exposure  to  cold  and  wet,  com- 
plained of  a  feeling  of  weight  and  weakness  in  the  legs  ;  the  temperature 
rose  to  103° ;  the  loss  of  power  gradually  became  complete  in  the  legs 
and  spread  to  the  arms,  without  loss  of  sensation.  At  the  end  of  the 
second  day  there  was  difl5.culty  in  swallowing,  in  articulation,  and  in 
breathing,  and  death  seemed  near.  Ergotin  was  given  every  hour, 
and  during  the  night  the  patient  took  twenty  grains.  In  the  morning 
the  bidbar  symptoms  were  better,  the  arms  stronger,  and  there  was  a 
trace  of  motoi'  power  in  the  legs.  The  patient  rapidly  improved,  and 
at  the  end  of  a  week  was  well.  If  swallowing  becomes  difficult,  care 
must  be  taken  to  administer  a  sufficient  amount  of  nourishment,  either 
by  the  reotum  or  by  the  nasal  tube.  A  few  cases  have  been  recorded 
in  persons  who  had  had  syphilis,  in  whom  arrest  of  the  disease  followed 
the  administration  of  iodide  of  mercury. 

The  chief  lesson  of  modem  research  is  certainly  that  we  must  look 
for  the  means  of  effective  treatment  to  the  neutralisation  of  the  toxic 
influence  on  which  the  malady  apparently  depends,  but  at  present 
nothing  has  been  ascertained  regarding  antidotal  agents  available  in 
the  acutely  active  stage  of  these  toxsemic  states.  It  is,  however,  pro- 
bable that  mercury  is  capable  of  doing  good,  and  that  when  the  causa- 


DIVERS*    PARALYSIS.  433 

tion  IB  o'bscure,  and  tlie  disease  develops  slowly  enougli  to  permit 
mercttrial  treatment,  it  is  "wise  to  adopt  this  irrespective  of  the  fact  of 
preceding  syphilis.  In  cases  that  follow  traumatic  lesions,  especially 
if  there  have  been  any  indications  of  septicsemia,  full  doses  of  per- 
chloride  of  iron  offer,  I  believe,  the  best  means  of  destroying  the 
activity  of  the  blood-state.  The  general  and  local  treatment  should 
be  the  same  as  in  acute  myelitis. 


PAEALTSIS  FROM  LESSENED   ATMOSPHETMC  PRES- 
SURE; DIYERS'  PARALYSIS;  CAISSON    DISEASE. 

Divers,  and  especially  those  who  work  in  caissons,  at  such  a  depth 
beneath  water  that  they  are  exposed  to  considerable  pressure,  may  be- 
come paralysed  soon  after  their  return  to  the  surface.*  Apoplectic 
attacks  and  hemiplegia  also  sometimes  occur,  sometimes  transient 
mental  disturbance,  or  deafness,  or  bleeding  from  the  nose,  but  para- 
lysis of  the  legs  is  by  far  the  most  common  effect,  and  it  is,  therefore, 
clear  that  the  spinal  cord  suffers  in  greater  degree  than  any  other  part 
of  the  nervous  system.  Miners  have  been  said  to  suffer  also,  but  this  is 
doabtful,  because  a  pressure  equal  to  at  least  an  additional  atmosphere 
seems  necessary  for  the  production  of  the  symptoms.  Most  of  the 
subjects  of  the  disease  have  worked  at  a  depth  of  from  forty  to 
ninety  feet  below  the  surface  of  water,  and  under  a  pressure  of  two 
to  four  atmospheres.  In  the  extensive  works  involved  in  laying  the 
foundations  of  bridges,  in  which  many  men  have  been  employed,  a 
considerable  proportion  of  the  workers  have  suffered  in  some  measure ; 
but  severe  degrees  of  affection  are  rare,  because  the  conditions  under 
which  they  occur  can  be  avoided.  They  only  occui-  in  those  who  have 
been  exposed  for  more  than  a  certain  time,  which  is  the  shorter  the 
greater  the  pressure.  At  a  depth  of  ninety  feet  beneath  the  surface 
immtmity  from  severe  symptoms  (and  commonly  from  all  symptoms) 
is  obtained  by  reducing  the  periods  of  work  to  an  hour.  The  danger 
,is  greater  in  those  who  have  had  several  previous  periods  of  work  on 
the  same  day,  and  especially  if  slight  symptoms  have  been  experienced 
after  one  of  these.  It  is  also  greater  in  those  unaccustomed  to  the 
conditions.  I  have  met  with  one  case  of  hemiplegia  in  a  diver  after 
only  half  an  hour's  work  in  a  well  at  ninety- six  feet,  but  the  man  had 
been  down  twice  before  with  only  intervals  of  rest  of  half  an  hour, 
and  the  attack  passed  off  in  the  course  of  an  hour.  In  another  case 
paraplegia  occurred  after  working  for  an  hour  under  only  fifty  feet  of 

*  See,  on  this  subject,  Babington  and  Cuthbert,  '  Dubl,  Quart.  Journ.,'  1863, 
p.  312  (cases  at  Londonderi-y) ;  Eads,  'Med.  Times  and  Gaz.,'  1871,  p.  291  (cases- 
at  St.  Louis) ;  Leyden,  *  Arch.  f.  Psychiatrie,'  ix.  Heft  2  j  and  Moxon,  '  Lancet,* 
1881,  ii,  529. 

VOL.  I.  28 


434  SPINAL    CORD. 

water,  but  tlie  man  liad  worked  on  tlie  preceding  day  at  a  depth  of 
one  hundred  and  twenty  feet  below  the  surface.  It  is  evident,  there- 
fore, that  the  power  of  resisting  the  dangerous  injEuence  varies,  and 
that  predisposition  to  suffer  may  be  caused  by  the  conditions  which 
also  induce  an  attack. 

It  is  not  during  exposure  to  the  abnormal  conditions,  but  on  return- 
ing to  the  normal  atmospheric  pressure,  that  the  symptoms  come  on ; 
often  immediately,  and  always  within  half  an  hour  or  at  most  an  hour 
of  the  return  to  the  surface.  The  onset  is  usually  preceded  by  other 
symptoms,  and  especially  by  pains  in  the  ears  and  in  the  joints.  The 
latter  are  very  common,  and  occur  after  a  much  slighter  degree  of 
exposure  than  is  needed  to  produce  paralysis.  They  are  felt  chiefly  in 
the  larger  joints,  and  may  continue  for  days  and  even  longer.  Yery 
rarely  there  is  also  swelling  of  the  joints. 

The  deafness  occasionally  observed  has  been  usually  bilateral,  often 
persisting  in  one  ear,  but  passing  away  in  the  other.  It  has  even  been 
attended  by  severe  vertigo.  The  severer  degree  has  been  ascribed  to 
haemorrhage  into  the  labyrinth,  but  no  pathological  facts  are  at  present 
available. 

It  is  important  to  note  the  conditions  under  which  the  symptoms 
come  on.  ISTot  only  do  those  who  are  unused  to  such  work  seem 
especially  liable  to  suffer,  but  there  are  also  considerable  individual 
variations  in  the  degree  of  liability. 

Paraplegia  or  hemiplegia  may  occur,  but  the  former  is  far  more 
common.  The  onset  of  the  paralysis  is  sudden.  The  legs  feel  heavy 
and  are  found  to  be  weak,  and  in  a  few  minutes  the  patient  is  unable 
to  move  them.  Sensation  is  often  lost  as  well  as  motion,  and  ia  all 
severe  cases  the  sphincters  are  affected.  The  arms  are  seldom 
involved.  In  sHght  cases  the  loss  of  power  is  incomplete,  and  one 
leg  may  be  more  affected  than  the  other.  The  impairment  of  sensa- 
tion is  often  imperfect  or  irregular.  If  the  paralysis  is  incomplete 
the  power  may  return  in  the  course  of  a  few  days,  but  in  severe  cases 
the  palsy  usually  lasts  for  weeks  or  even  months,  and  it  may  be 
permanent.  Death  may  occur  at  the  end  of  a  week  or  more,  in  the 
same  way  and  from  the  same  causes  as  in  acute  myelitis.  When  the 
paralysis  is  hemiplegic  it  is  generally  moderate  in  severity  and 
transient,  passing  off  in  a  few  hours  or  days.  It  may  be  even  still 
more  brief,  as  in  the  case  referred  to  above,  in  which  a  diver,  after 
half  an  hour's  work  ninety-six  feet  below  the  surface,  suddenly  felt 
tingling  across  his  loins,  and  that  his  right  arm  and  leg  were  almost 
powerless  ;  in  half  an  hour  he  had  recovered  sufficiently  to  walk  home- 
If  cerebral  symptoms  are  more  intense,  they  are  generally  very  severe. 
with  sudden  loss  of  conscioTisness,  continuing  as  deep  coma,  irregulai 
I)reathing,  and  indications  of  cardiac  pavT  lysis.  Such  cases  Tisually 
«nd  in  death  in  the  course  of  a  few  hours. 

Pathology. — The  most  probable  explanation  of  these  cases  ascribes 
them  to  the  escape  from  the  blood  of  gases,  with  which  it  has  become 


divers'  paralysis.  435 

charged  during  the  exposure  to  the  high  pressure.  It  is  certain  that 
a  great  excess  of  gas  must  be  dissolved  in  the  blood  during  the  expo- 
sure, and  that  the  amount,  at  least  of  oxygen  and  of  carbonic  acid, 
contained  in  the  blood  when  the  person  emerges  from  the  caisson 
must  be  vastly  greater  than  normal.  It  must,  moreover,  be  greater 
the  longer  the  exposure,  since  the  absorption  of  the  excess  vsdll  be 
a  gradual  process,  taking  place  through  the  lungs  in  respiration,  but 
it  will  be  more  rapid  as  the  pressure  is  greater.  These  conditions 
agree  with  observed  facts.  After  returning  to  the  normal  atmospheric 
pressure,  the  excess  of  gas  probably  passes  off  gradually  by  the  lungs 
in  most  instances ;  but  if  extreme,  gas  may  escape  from  the  blood 
within  the  body.  The  occurrence  of  this  has  been  proved  experimen- 
tally (Hoppe-Seyler  and  P.  Bert).  G-as,  so  escaping,  is  no  doubt 
quickly  reabsorbed,  but  must  exert  a  pressure  capable  of  arresting  the 
function  of  the  structures  of  the  nervous  system.  If  abundant,  it 
may  conceivably  rupture  these.  Further,  the  special  effect  on  the 
nerve-centres  may  be  connected  with  the  position  of  these  within 
cavities  that  are  practically  closed.  These  conditions,  coupled  with 
the  extremely  circuitous  course  of  the  blood  from  the  cord,  may 
explain  the  incidence  of  the  effects  on  the  nervous  system. 

The  few  ascertained  facts  harmonise  with  this  pathology.  Such 
escape  of  gas  has  been  proved  to  result  from  diminution  of  the 
atmospheric  pressure.*  In  many  cases  no  visible  lesions  have  been 
found,  and  it  is  obvious  that  gas,  widely  effused,  may  exert  dangerous 
pressure,  and  yet  may  be  speedily  removed,  so  that  no  indication  may 
remain  of  the  cause  that  has  completely  arrested  function. 

It  was  once  thought  that  the  symptoms  were  due  to  local  haemor- 
rhages, but  extravasations  are  seldom  met  with,  even  in  the  results  of 
experiment,  and  it  is  certain  that  they  take  but  a  trifling  part  in  the 
production  of  symptoms.  The  only  positive  pathological  observation 
agrees  with  the  opinions  above  stated.  Ley  den  f  foimd  (in  a  case  of 
characteristic  paraplegia)  small  irregular  fissures  in  the  mid-dorsal 
region,  chiefly  within  the  posterior  and  hinder  parts  of  the  lateral 
column.  The  fissures  were  filled  with  round-cells,  but  contained  no 
red  blood-corpuscles,  and  from  their  well-defined  edges  they  were  cer- 
tainly not  produced  by  the  infiltration  of  the  cells  found  within  them. 
The  only  explanation  that  is  satisfactory,  or  in  any  harmony  with 
their  features,  is  that  they  were  produced  by  the  sudden  escape  of 
gas,  and  were  afterwards  occupied  by  the  round-cells. |  The  physical 
firmness  of  the  cord  is  far  less  in  the  dorsal  region  than  in  the  en- 
largements ;  and  probably,  as  Ley  den  suggests,  this  is  the  reason  why 
the  dorsal  region  suffers  structural  damage  from  the  escape  of  gas 

*  Hoppe-Seyler,  'MuUer's  Archiv,'  1887;  P.  Bert,  'Comptes  Rend.,'  1871-2. 

t  •  Arch.  f.  Psych.,'  ix.  Heft  2. 

J  The  clinical  condition  of  transient  hemiplegia,  which  has  been  observed  in 
empyema  as  occurring  during  irrigation,  and  thought  by  some  to  be  the  result 
of  air  or  gas  embolism,  may  be  of  similar  nature. 


436  SPINAL    COED. 

more  than  otlier  parts.  It  is  evident,  however,  that  such  escape  maj 
occur  in  the  brain,  for  instance,  even  to  a  fatal  degree,  and  leave  no 
traces  unless  it  occurs  so  rapidly  as  to  cause  laceration  of  the  tissue. 
The  absence  of  haemorrhage  of  the  ordinary  chara.cter  may  be  further 
explained  by  the  fact  that  the  effused  air  will  resist  the  effusion  of 
blood.  Some  other  anomalous  features,  such  as  the  transient  cha- 
racter of  the  symptoms  in  some  cases,  are  also  explained  by  the  peculiar 
nature  of  the  lesion,  which  may  exert  a  wide-spread  and  considerable 
influence,  and  in  a  short  time  may  have  vanished  by  the  reabsorp- 
tion  of  the  compressing  air.  Haemorrhage  must  be  regarded  as  an 
accidental  consequence,  and  the  production  of  such  fissures  as  were 
found  by  Leyden  may  not  be  a  necessary  part  of  the  lesion,  even  in 
grave  cases  The  mechanical  effects  of  the  gas  may  be  considerable, 
and  arrest  the  function  of  the  nerve-elements,  without  the  passage  of 
the  gas  outside  the  capillary  vessels. 

The  fact  that  the  spinal  cord  suffers  more  than  other  organs  may 
be  partly  due  to  the  plexuses  through  which  the  blood  can  return  only 
slowly  to  the  lungs,  where  its  relief  from  the  surcharge  of  gas  is  effected. 
The  same  considerations  apply  to  the  brain.  In  both  organs  the 
escape  of  air  is  more  instantly  disastrous  than  elsewhere,  and,  indeed, 
may  not  only  occur,  but  act  in  a  peculiar  way,  on  account  of  the  posi- 
tion of  the  organs  within  chambers  that  are,  to  a  large  degree,  closed. 
The  conditions  are  too  complex  to  permit  us  to  follow  them  in  detail, 
or  analyse  fully  the  mechanisms  that  determine  the  effect  on  the 
nerve-centres.  The  escape  of  the  excess  of  gas  in  the  lungs  can  only 
take  place  gradually,  as  successive  quantities  of  the  surcharged  blood 
pass  through  the  capillaries  of  the  air-cells.  If,  in  consequence  of  the 
slowness  of  the  circulation  in  the  cord,  any  escape  of  air  occurs  in  its 
capillaries,  the  effect  of  this  will  be  still  further  to  hinder  the  local 
circulation,  and  to  favour  the  further  escape  of  gas.  In  this  way  we 
are  able  to  discern  something  of  the  mechanism  which  determines  the 
special  affection  of  the  spinal  cord.  The  less  the  external  support  the 
less  will  be  the  resistance  to  the  escape  of  gas,  and  the  incidence  of 
the  lesion  on  the  posterior  part  of  the  cord  may  be,  in  part  at  least, 
determined  by  the  adoption  of  the  recumbent  posture  when  the  first 
symptoms  are  perceived. 

At  the  onset,  when  the  first  symptoms  are  experienced,  it  is  probable 
that  a  return  to  a  greater  degree  of  pressure  might  arrest  the  develop- 
ment of  the  mischief.  It  is  certain  that,  in  all  cases  of  exposure  to  a 
high  degree  of  pressure,  the  transition  to  the  normal  pressure  should 
be  made  gradually.  When  developed  symptoms  indicate  that  a 
definite  lesion  of  the  cord  has  occurred,  the  further  treatment  must 
be  conducted  on  the  same  principles  as  in  acute  myelitis — the  morbid 
process  that  has,  in  fact,  been  set  up. 


H^MOEEHAGE.  487 


H^MOERHAGE   INTO   THE   SPINAL   CORD; 

H^MATOMYELIA. 

Primary  hsemorrliage  into  the  spinal  cord,  sufficient  to  cause  sym- 
ptoms, is  a  very  rare  disease,  and  it  is  even  more  rare  than  is  suggested 
by  the  cases  now  and  then  recorded  as  such.  We  have  ah-eady  seen 
that  haemorrhage  may  accompany  myelitis ;  a  considerable  extravasa- 
tion may  occur  when  the  inflammation  is  only  commencing,  during  the 
state  of  congestion,  and  while  the  symptoms  are  slight.  Such  cases 
are  easily  mistaken  for  primary  haemorrhage.  The  risk  of  error  is  not 
always  removed  by  pathological  examination.  Inflammation  results 
from  haemorrhage,  and  when  indications  of  myelitis  are  found  about 
a  clot,  it  may  be  impossible  to  say  whether  these  are  primary  or 
secondary.  It  is  probable  that  many  cases  of  secondary  myelitic 
haemorrhage  have  been  regarded  and  described  as  primary,  and  it  is 
possible  that  a  few  cases  of  primary  haemorrhage  have  been  regarded 
as  secondary.  One  writer,  Hayem,*  goes  so  far  as  to  deny  the  occur- 
rence of  primary  non-traumatic  haemorrhage ;  but  such  an  exclusive 
view  is  unwarranted.  It  is  certain,  however,  that  the  history  of 
primary  haemorrhage  has  been  largely  written  from  uncertain  data, 
and  will  need  extensive  revision  when  a  sufficient  number  of  exact 
observations  have  accumulated.  It  is  probable  that  the  causal 
influences  constitute  the  surest  guide.  When  haemorrhage  occurs 
under  conditions  which  usually  induce  myelitis,  the  extravasation 
must  be  regarded  as  the  result  of  the  vascular  disturbance  of  com- 
mencing inflammation. 

Etiologt. — The  rarity  of  haemorrhage  into  the  cord  is  especially 
great  in  comparison  with  the  frequency  of  haemorrhage  into  the  brain. 
The  difference  probal^ly  depends  on  the  tortuous  and  long  course  of 
the  arterial  path  to  the  cord,  whereby  the  vessels  are  preserved  from 
the  high  pressure  which  is  the  chief  cause  of  the  degeneration,  dilata- 
tion, and  rupture  of  the  cerebral  arteries.  Miliary  aneurisms  are  not 
found  within  the  spinal  cord.  Haemorrhage  is  far  more  common  in 
males  than  in  females.  It  may  occur  at  any  age,  and  has  been  met 
with  in  young  children,  even  so  early  as  seven  months,t  while  some  of 
the  subjects  have  been  in  advanced  life ;  but  it  is  most  common 
between  twenty  and  forty,  i.  e.  during  the  first  half  of  adult  life. 
Many  cases  occur  in  young  adults  apart  from  injury  or  obvious 
exciting  cause,  and  apart  also  from  initial  myelitis,  which  accounts, 
however,  for  some  of  the  cases.  It  has  been  met  with  as  a  conse- 
quence of  a  haemorrhagic  tendency,  as  in  a  case  in  which  it  succeeded 

*  '  Des  H^morragies  intra-rachidienues/  Paris,  1872. 

+  Clifford  Allbutt, '  Lancet,'  1870,  vol.  ii,  p.  84.  Numerous  haemorrhages  were 
observed  in  the  grey  matter  of  the  lumbar  and  cervical  enlargements  (probably 
polio-myelitic,  but  occurring  the  day  after  a  fall)  in  a  child  of  four  by  ChafEey 
('  Path.  Trans.,'  1885). 


438  SPINAL   CORD. 

severe  epistaxis,  to  whicli  the  patient,  a  young  man  of  twenty- four, 
was  liable.* 

Of  immediate  causes,  injury  is  the  most  frequent,  especially  falls 
whicli  involve  a  severe  concussion  of  the  spine ;  the  spinal  column  may 
or  may  not  be  injured  at  the  same  time.  Over-exertion  and  exposure 
to  cold  have  in  rare  cases  preceded  the  onset.  Chronic  alcoholism  and 
sexual  excess  have  been  thought  to  predispose.  In  one  case  within 
my  knowledge,  an  extensive  hsemorrhage  into  the  grey  substance  ai 
the  top  of  the  lumbar  enlargement  resulted  from  coitus  four  times 
repeated,  the  symptoms  commencing  suddenly  during  the  fourth  act. 
Minute  extra,vasations  are  often  found  after  death  from  diseases  which 
interfere  with  respiration  and  cause  venous  congestion,  and  they  are 
especially  frequent  in  maladies  which  at  the  same  time  cause  func- 
tional excitement  of  the  cord,  as  tetanus  and  all  severe  convulsions. 
They  cause  no  symptoms,  and  are  probably  produced  during  the  last 
moments  of  life.  They  have  been  termed  "  accessory."  The  diseases 
of  the  cord  that  lead  to  secondary  hsemorrhage  are  chiefly  inflamma- 
tion, tumours,  and  cavities  in  the  cord.  The  last-named  condition  is 
especially  important.  It  is  probable  that  hsemorrhage  occurs  more 
readily  if  the  spinal  cord  contains  a  congenital  cavity  or  fissure,  due 
to  an  arrest  of  development,  and  surrounded  by  embryonal  neuroglial 
tissue.  Hence  this  condition  will  be  fotmd  in  disproportionate  fre- 
quency in  cases  of  hsemorrhage. 

PATHOLOGICA.L  Anatomy. — The  minute  extravasations  just  men- 
tioned, as  met  with  after  asphyxial  and  convulsive  diseases,  are  found 
in  both  the  grey  and  white  substance,  but  especially  in  the  former. 
They  are  usually  microscopic,  or  visible  to  the  naked  eye  as  minute 
red  points,  distinguishable  from  distended  vessels  only  by  their 
slighter  resistance  to  a  stream  of  water.  The  extravasation  may 
occupy  the  perivascular  sheath,  or  the  cavity  in  which  the  vessel  lies, 
or  extend  between  the  nerve-elements.  The  larger  non-traumatic 
haemorrhages,  which  cause  symptoms,  always  begin  in  the  grey 
substance,  and  are  often  confined  to  it,  extending  into  the  white 
columns  only  when  large  in  size.  The  vessels  of  the  grey  substance 
are  more  numerous  than  those  of  the  white,  have  less  external  support, 
and  probably  undergo  more  considerable  changes  in  state.  The 
effusion  forms  a  cavity  in  the  cord,  sometimes  rounded,  sometimes 
irregular  in  transverse  section,  and  half  an  inch  or  more  in  vertical 
extent.  The  cord  is  enlarged  at  the  seat  of  the  hsemorrhage,  and 
this  may  be  visible  externally  as  a  dark  swelling  the  size  of  a  nut  or 
a  bean.  Very  rarely  the  hsemorrhage  tears  the  layer  of  cord  which 
limits  it,  and  blood,  usually  only  in  small  quantity,  escapes  into  the 
membranes.  The  tissue  adjacent  to  the  clot  is  usually  broken  down, 
stained,  and  softened,  and  inflammatory  changes  may  be  recognised 
in  it  with  the  microscope,  just  as  in  the  neighbourhood  of  hsemor- 
rhages  into  the  brain.  As  in  the  latter,  the  effused  blood  slowly 
*  Sinclair, '  Lancet/  1885,  ii,  p.  1043. 


h^moeehagb.  439 

undergoes  changes  in  tint,  becoming  first  rusty  and  then  3'ellow,  and 
ultimately  a  cyst  may  remain.  Several  extravasations  may  co-exist, 
usually  in  the  same  part  of  the  cord.  In  contrast  to  this  focal 
Jisemorrhage  there  may  be  an  infi.ltration  of  the  grey  matter  with 
punctiform  extravasations,  which  may  appear,  until  closely  examined, 
to  be  a  single  haemorrhage.  The  tissue  bet"ween  these  small  extra- 
vasations is  broken  down.  It  is  probable  that  this  form  is  always 
secondary  to  myelitis,  as  in  the  case  mentioned  in  the  foot-note  on 
p.  437.  In  such  secondary  cases  a  careful  microscopical  examination 
usually  reveals  indications  of  inflammation  much  more  extensive  than 
the  area  affected  by  the  haemorrhage.  A  growth  into  which  hsemor- 
rhage  occurs  is  usually  a  glioma,  unaffected  parts  of  which  will  be 
found  in  the  neighbourhood  of  the  extravasation.  The  cavities  in  the 
cord  into  which  blood  may  escape  are  sometimes  of  considerable 
vertical  extent.  I  have  known  a  fissure  in  the  posterior  column  to  be 
filled  with  blood  through  almost  the  whole  length  of  the  cord.  This 
condition  thus  favours  both  the  occurrence  and  the  extension  of 
haemorrhage. 

Symptoms. — Slight  symptoms,  "  prodromata,"  have  been  obseiwed 
in  some  cases,  chiefly  in  the  form  of  trifling  sensory  disturbance, 
tingling,  &c.,  in  the  Hmbs  afterwards  paralysed.  They  have  existed 
for  a  few  hours  or  days,  or  even  for  two  or  three  weeks  before  the 
onset.  It  is  probable,  however,  that  these  have  been  cases  of 
secondary  myelitic  haemorrhage,  and  that  there  are  no  premonitory 
symptoms  in  primary  extravasations.  The  actual  onset  is  always 
sudden  ;  the  symptoms  attain  a  considerable  degree  in  the  course  of 
a  few  minutes,  but  they  sometimes  increase  during  one  or  two  hours, 
when  the  haemorrhage  is  from  a  small  vessel  and  slowly  increases  in 
size — probably  augmented  by  the  rupture  of  other  vessels  which  are 
torn  in  the  tissue  lacerated  by  the  blood.  Occasionally  there  has 
been  transient  loss  of  consciousness  without  any  cerebral  lesion, 
probably  from  the  upward  influence  of  the  shock.  Earely  the  onset 
has  been  by  a  series  of  sudden  augmentations  of  the  symptoms. 
Sometimes  the  symptoms  come  on  during  sleep.  The  suddenness  of 
the  onset  is  the  characteristic  of  the  disease. 

The  symptoms  which  thus  develop  vary  according  to  the  seat  and 
extent  of  the  extravasation.  In  the  majority  of  cases  there  is  para- 
plegia, coiuplete  motor  and  sensory  paralysis  up  to  the  level  of  the 
lesion,  with  loss  of  power  over  the  sphincters.  Pain  commonly  but 
not  invariably  accompanies  the  sudden  palsy ;  it  may  be  felt  in  the 
spine  or  in  the  sacrum,  round  the  trunk,  at  the  front  of  the  thorax, 
or  at  the  epigastrium,  sometimes  seeming  to  pass  thence  through  the 
trunk  to  the  spine,  or  it  may  be  felt  in  the  legs.  If  in  the  spine  it 
is  local,  and  does  not  extend  through  a  considerable  length  of  the 
spine,  as  in  meningeal  haemorrhage,  and  there  is  not  the  initial  spasm 
and  rigidity  which  characterise  the  latter  disease.  This  pain  may 
precede  the  palsy,  even  for  half  an  hour  or  an  hour ;  it  begins  suddenly, 


440  SPINAL   CORD. 

and  is  n(>  doubt  due  to  tlie  first  sliglii;  extravasation,  whicli  afterwards 
increases  (when  some  resistance  yields),  and  effects  the  compression 
tliat  produces  the  paralysis.  Thus  a  girl  aged  fifteen  was  seized 
with  sudden  sharp  pain,  referred  to  the  sternum  at  the  mammary 
level,  and  to  the  corresponding  region  of  the  spine ;  this  continued 
for  half  an  hour,  when  the  legs  suddenly  became  powerless  and  insen- 
sitive. It  is  highly  probable  that  when  there  is  such  initial  medial 
or  bilateral  pain  the  haemorrhage  occurs  in  the  central  region  of  llie 
grey  matter,  perhaps  from  one  of  the  commissui*al  or  anastomotic 
arteries  (see  p.  227),  and  that  the  pain  is  due  to  the  irritation  of  the 
fibres  of  the  posterior  commissure,  in  which  the  paths  for  pain  cross 
the  middle  line.  The  spinal  column  may  be  tender  opposite  the 
affected  spot.  TJsiially  the  paralysed  muscles  are  relaxed.  Some- 
times they  are  the  seat  of  early  clonic  contractions,  or  these  may  come 
on  a  few  days  after  the  onset.  When  the  hsemorrhage  is  in  the 
cervical  region  all  the  limbs  are  powerless  ;  one  arm  is  often  affected 
before  the  other.  The  state  of  reflex  action  varies  according  to  the 
seat  of  the  disease ;  if  it  is  at  first  abolished  it  quickly  returns  in  the 
legs  (unless  the  hsemorrhage  is  in  the  lumbar  enlargement),  and  it 
soon  becomes  excessive  unless  destroyed  by  secondary  myehtis — a 
not  unfrequent  consequence.  In  one  case  there  was  a  remarkable 
initial  increase  of  myotatic  irritability  a  few  hours  after  the  onset, 
probably  irritative,  and  quickly  giving  place  to  loss.*  Vaso-motor 
and  trophic  changes  in  the  skin  are  common  and  often  intense  ;  there 
is  often  vascular  dilatation ;  bedsores  readily  form,  and  cystitis  may 
result.  Frequently  the  secretion  of  sweat  is  increased  for  a  time. 
The  temperature  is  normal  at  the  onset,  but  it  generally  rises  in  the 
course  of  a  few  days  from  secondary  inflammation  in  the  cord. 

The  palsy  developed  at  the  onset  usually  continues  for  a  week  or 
ten  days,  although  the  pain  may  lessen.  The  symj^toms  do  not  always 
increase  during  the  stage  of  inflammation,  perhaps  because  this  only 
involves  the  structures  which  are  already  impaired  by  pressure  ;  but  if 
the  patient  has  been  brought  near  to  death  by  the  primary  haemor- 
rhage, the  secondary  inflammation  may  end  hfe.  Occasionally,  more- 
over, symptoms  of  an  ascending  or  descending  myehtis  may  come  on, 
and  the  former  may  cause  death  by  its  interference  with  the  muscles 
of  respiration.  This  extension  is  greatest  probably  in  cases  of 
myelitic  hsemorrhage,  in  which  the  extravasation  is  merely  an  incident 
in  the  course  of  a  commencing  inflammation,  but  it  seems  also  to  occur 
in  cases  of  primary  hsemorrhage.  The  slow  extension  upwards  of  the 
symptoms  during  the  first  week  may  be  very  distinct ;  and  the  occur- 
rence of  descending  inflammation  may  be  shown  by  the  loss  of  reflex 
action,  and  by  the  failure  of  electric  irritability  in  the  muscles,  sometimes 
only  in  those  supplied  from  the  upper  part  of  the  lumbar  enlargement. 

The  symptoms  pass  into  a  chronic  stage,  improvement  being  usually 
slow.     Some  lasting  loss  of  power  remains  in  most  cases,  and  there  ia 

*   Sinclair,  loc,  cit. 


H^MOERHAGE.  441 

often  some  permanent  muscular  wasting  on  account  of  the  frequency 
with  whicli  the  haemorrhage  is  in  the  cervical  or  lumbar  grey  matter. 
Occasionally  there  is  rapid  recovery  up  to  a  certain  point ;  a  haemor- 
rhage of  small  size  may  abolish  conduction  in  the  white  columns  by 
the  suddenness  with  which  it  compresses  them,  and  the  effects  of  the 
pressure  may  quickly  pass  away,  while  those  remain  that  are  due  to 
the  destruction  of  the  grey  matter. 

DiACNOsis. — The  diagnosis  rests  on  the  actually  sudden  onset  of 
the  symptoms,  and  on  the  occurrence  of  pain,  in  a  limited  region,  as 
part  of  these.  The  former  is  evidence  of  a  vascular. lesion  (rupture 
or  closure),  and  the  latter  shows  an  acute  irritation  of  the  nerve- 
elements,  such  as  may  be  produced  by  their  laceration,  but  not  by 
mere  deprivation  of  blood.  It  must  be  remembered,  however,  that 
we  cannot  assume  that  symptoms  which  come  on  during  the  night's 
sleep  are  of  sudden  onset.  Neglect  of  this  consideration  sometimes 
causes  a  mistake  in  diagnosis.  The  mode  of  onset  is  a  sufficient 
distinction  from  all  other  organic  diseases,  except  haemorrhagic 
myelitis  and  meningeal  haemorrhage.  The  former  (really  a  variety 
of  haemorrhage)  is  distinguished  by  the  existence  of  slight  symptoms 
before  the  sudden  attack.  We  are  not  justified  in  regarding  as 
primary  haemorrhage  any  case  in  which  premonitory  symptoms 
existed  for  more  than  a  few  minutes,  unless  such  symptoms  were  so 
pronounced  and  sudden  in  onset  that  they  might  have  been  due  to  a 
definite  extravasation,  afterwards  increasing.  Initial  fever  (within 
the  first  six  hours)  always  makes  myehtis  probable,  provided  there 
is  no  other  cause  for  it.  The  distinction  from  meningeal  haemorrhage 
has  been  mentioned  in  the  account  of  the  symptoms,  and  in  the 
description  of  that  disease. 

Prognosis. — In  all  cases  in  which  the  symptoms  are  considerable  in 
degree  or  wide  ia  range,  the  danger  to  life  is  great,  and  remains  great 
until  they  begin  to  subside.  The  prognosis  is  better  when  the  disease 
is  in  the  dorsal  region  than  when  it  is  in  the  enlargements,  for  the 
same  causes  as  influence  the  prognosis  ia  myelitis.  It  is  better  when 
sensation  retiims  in  the  course  of  a  few  days,  but  if  the  enlargements 
are  affected  other  sources  of  danger  remain  considerable.  Early 
trophic  changes  also  render  the  prognosis  worse,  for  they  show  an 
intensity  of  degree  that  may  involve  grave  danger.  After  the  onset  is 
over,  the  forecast  must  be  guided  by  the  general  principles  that  deter- 
mine the  prognosis  in  acute  myehtis. 

Treatment. — The  treatment  of  haemorrhage  into  the  subst-ance  of 
the  cord  is  the  same  as  that  of  haemorrhage  into  the  membranes 
(p.  339)  The  measures  requisite  are  few,  simple,  and  all-important. 
Absolute  rest  and  the  prone  position  are  to  be  secured  bef  oi-e  anything 
else  is  thought  of.  Ice  should  be  applied  to  the  spine  over  the  seat  of 
the  haemorrhage.  The  bowels  should  be  opened  freely,  and  fidl  doses 
of  ergot  or  ergotin  may  be  given :  5ss  of  the  liquid  extract  or  five 
grains  of  ergotin  may  be  given  by  the  mouth,  or  three  grains  of  ergotin 


442  SPINAL   COED. 

may  be  injected  iinder  tlie  stin,  and  tlie  dose  may  be  repeated  two  oi 
three  times,  at  intervals  of  two  hours.  A  few  large  doses  are  probably 
more  effectual  than  smaller  doses  continued  for  a  longer  time,  since 
tbe  baemorrliage  probably  does  not  go  on  for  long.  The  after-treat- 
ment must  be  that  for  myelitis.  The  disease  is  one  of  those  in  which 
most  of  the  good  that  can  be  done  by  treatment — and  in  few  diseases 
is  the  opportunity  more  urgent— rests  with  those  in  whose  hands  the 
patient  is  immediately  after  the  onset. 


DEGENERATIONS   OF   THE   SPINAL    COED. 

A  large  and  important  class  of  diseases  of  the  spinal  cord  consists  of 
those  in  which  there  is  a  slow  degeneration  of  the  nerve-elements,  with 
an  overgrowth  of  connective  tissue,  and  in  which  structures  are  affected 
that  have  a  common  function,  while  others  that  have  a  different 
function  escape  even  when  they  are  adjacent  to  the  elements  that  are 
diseased.  Affecting  thus  functional  "  systems,"  they  are  termed 
'•  system  diseases."  This  term  has  been  used  in  several  senses,  more 
or  less  special  (even  based  on  the  developmental  relations  of  the 
structures),  and  hence  the  question  whether  a  given  malady  is  a 
"  system  disease  "  or  not,  is  one  to  which  various  answers  have  been 
given.  The  term  is  here  used  in  its  widest  and  simplest  sense,  aa 
meaning  an  affection  of  the  structures  that  have  a  function  either  the 
same,  or  so  far  allied  that  they  work  together  as  parts  of  one  system, 
distinguishable  as  such  from  other  sets  of  structures.  A  "  system 
disease  "  may  involve  all  the  structures  of  the  system,  or  only  some  or 
one  of  them  ;  its  chief  distinction  is  from  a  random  disease  that 
involves  structures  irrespective  of  function — merely,  for  instance, 
because  they  are  contiguous,  or  supplied  by  the  same  artery. 

In  "  system  diseases  "  the  primary  change  is,  as  a  rule,  in  the 
nerve-elements,  and  the  overgrowth  of  interstitial  tissue  is  secondary. 
The  process  is  analogous  to  that  which  occurs  in  the  secondary 
degeneration  of  nerve-fibres,  in  which  the  first  change  is  certainly  in 
the  nerve-elements,  the  destruction  of  which  is  followed  by  an  over- 
growth of  nuclei  and  supporting  tissue,  amounting  ultimately  to 
a  "  sclerosis,"  as  it  is  termed.  The  process  is  by  some  regarded  as  a 
chronic  parenchymatous  inflammation,  an  inflammation  beginning  in 
the  proper  functional  elements  of  the  organ,  but  it  seems  undesirable 
thus  to  widen  and  loosen  our  conception  of  inflammation ;  so  far  as 
the  process  is  concerned  the  question  is  one  of  name  rather  than  of 
nature. 

Another  question  of  much  greater  interest  is  the  relation  of  the  two 
elements  in  the  process,  the  wasting  and  the  growth,  the  atrophy  of  the 


DEGENERATION.  443 

nerve  tissue,  the  hypertroplij  of  tlie  connective  tissue.  The  failure  of 
nutrition  in  the  one  causes  an  increased  energy  of  nutrition  in  the 
other.  We  have  seen  this  relation  in  the  nerve-fibres.  The  degene- 
ration of  a  fibre  is  attended  by  an  active  growth  of  its  nuclei  and 
protoplasm.  The  nutrition  of  the  two  elements,  the  neural  and  the 
adventitial,  is  evidently  connected  in  the  closest  manner,  but  in  inverse 
course.  It  is  important  to  recognise  the  fact  that  the  process  of 
growth  of  the  connective-tissue  elements  is  an  active  manifestation  of 
nutritional  energy,  which,  when  once  excited,  may  be  to  some  extent 
independent  of  its  cause.  It  may,  when  very  active,  pass  beyond  its 
proportional  limits,  and  be  greater  than  corresponds  to  the  nerve 
atrophy  that  causes  it,  invading  adjacent  structures  as  if  an  inde- 
pendent process.  It  may,  when  very  rapid,  have  some  of  the  cha- 
racters of  an  interstitial  inflammation,  and  even  an  acute  inflamma- 
tion. Such  excess  is  quite  unusual ;  as  a  rule  the  secondary  process 
is  subordinate  and  proportioned  to  the  primary  change. 

These  degenerative  diseases  fall  into  certain  types  so  far  as  the  spinal 
cord  is  concerned,  but  they  are  subject  to  some  variety  of  combination 
even  there,  and  to  great  diversity  of  association  with  degenerative 
changes  elsewhere.  The  affection  of  the  cord  is  of  either  the  sensory 
or  the  motor  elements,  or  both.  The  type  of  the  former  is  tabes ;  of 
the  latter,  either  spastic  paraplegia  or  muscular  atrophy,  Recording  as 
the  lower  segment  of  the  motor  path  is  affected,  or  the  upper.  Other 
combinations  of  affection  will  be  better  understood  when  the  diseases 
are  described. 

The  degenerative  diseases  are  not  numerous,  but  they  are  of  great 
importance.  They  are,  degeneration  of  the  anterior  cornua  and  anterior 
root-fibres,  causing  muscular  atrophy ;  degeneration  of  the  posterior 
columns  and  posterior  root-fibres,  causing  locomotor  ataxy;  and 
degeneration  of  the  pyramidal  tracts,  causing  spastic  paraplegia,  a 
disease  the  exact  pathological  position  of  which  is  not  qnite  certain. 
These  degenerations  may  be  variously  combined,  and  some  combioa- 
tions  need  separate  description. 

Their  causes  are  various.  In  some  the  nutrition  of  the  nerve- 
elements  fails  soon  after  they  have  achieved  maturity,  and  this  is 
prone  to  occur  in  members  of  the  same  family.  In  other  cases,  which 
present  a  contrast  to  those  just  mentioned,  and  yet  are  allied,  there  is 
a  senile  degeneration,  apparently  in  consequence  of  less  vital  endur- 
ance in  these  elements  of  the  nervous  system  than  in  the  other 
structures  of  the  body.  In  such  cases  the  local  degeneration  merely 
anticipates  the  somatic  death  which  it  too  often  induces.  In  these  we 
must  assume  a,n  inherent  deficiency  of  vital  endurance  in  the  struc- 
tures that  fail.  A  similar  assumption  seems  to  be  the  only  explanation 
for  the  cases  in  which  degeneration  of  certain  elements  of  the  central 
nervous  system  occurs  in  middle  life,  between  thirty-five  and  fifty, 
usually  as  a  result  of  depressing  emotion,  or  of  some  influence  which 
lessens  the  general  nutrition.      Lastly,  we  are  confronted  with  a  large 


444  SPINAL   COilD, 

and  varied  class  of  cases,  in  whicli  similar  degenerations  seem  to  lesult 
from  toxic  agents  present  in  the  blood.  These  may  be  received  from 
without,  or  generated  within  the  system,  and  an  especially  potent  source 
of  such  toxins  seems  to  be  the  development  of  the  organisms  of  some 
specific  diseases. 


LOCOMOTOR  ATAXY;  TABES  DOESALTS. 

The  malady  thus  named  is  the  most  common  chronic  disease  of  the 
spinal  cord.  It  consists  in  a  degeneration  in  the  posterior  columns  of 
the  spinal  cord,  or  the  peripheral  sensory  nerves,  or  both.  According 
to  the  current  views,  it  is  the  expression  of  impaired  nutritional  in- 
fluence of  the  sensory  neurons  of  the  spinal  cord,  primarily  of  those 
related  to  the  afferent  muscle-nerves,  but  often  also  other  afferent 
nerve- systems,  including  those  from  the  skin.  The  nutritional  centre 
is  in  the  posterior  ganglia,  and  the  toxin  apparently  acts  first  on  the 
extremity  of  the  sensory  fibres,  and  also  on  those  that  pass  into  the 
cord.  The  effect  is  manifested,  when  considerable,  by  inco-ordination 
of  movement,  peculiar  pains,  defective  sensibility,  and  loss  of  the 
myotatic  irritability  (muscle-reflex  action),  of  which  the  knee-jerk  is 
the  most  convenient  manifestation.  When  slight  in  degree,  the  sym- 
ptoms may  be  limited  to  the  pains  and  the  loss  of  the  knee-jerk.  The 
name  "locomotor  ataxy"  was  given  to  the  disease  by  Duchenne; 
"tabes  dorsalis,"  or  "wasting  of  the  back,"  is  a  term  applied  by 
Hippocrates  to  certain  symptoms  supposed  to  be  due  to  venereal 
excess,  and  long  ago  limited  in  Germany  to  symptoms  believed  to 
depend  on  atrophy  of  the  spinal  cord.  It  then  included  all  chronic 
paraplegias,  but  was  further  restricted  to  this  disease  by  Eomberg. 
It  has  lately  obtained  wider  use  on  account  of  the  discovery  that  inco- 
ordination may  be  absent  when  the  disease  is  slight  in  degree. 

The  symptoms  vary  much  in  different  cases.  Besides  the  varieties 
thus  produced,  there  are  two  allied  affections  that  ought  not  to  be 
classed  with  it.  One  is  the  so-called  "  hereditary  ataxy."  The  other 
is  that  in  which  both  weakness  and  inco-ordination  co-exist  from  the 
first ;  "  ataxic  paraplegia  "  it  may  be  called.  Each  differs  from  other 
varieties  with  sufiicient  constancy  to  merit  separate  consideration. 
These  forms  are  not  included  in  the  following  account. 

HisTORT. — The  inco-ordination  of  movement  and  other  symptoms 
of  the  disease  were  frequently  noted,  during  the  first  third  of  this 
century,  in  cases  of  disease  of  the  spinal  cord,  but  such  cases  were  not 
distinguished  from  those  with  actual  loss  of  power.  The  inco-ordina- 
tion was  found  to  be  associated  with  disease  of  the  posterior  columns 
by  Stanley*  The  first  really  exact  account  of  the  disease  was  pub- 
*  '  Med.  Gazette,'  Feb.,  1840,  and  '  Med.-Chir.  Trans./  vol.  xxiii. 


LOCOMOTOR   ATAXY.  445 

lished  in  1847  by  Todd,*  who  distingtdslied  tlie  cases  with  inco-ordina- 
tion  and  without  weakness,  from  simple  paraplegia,  and,  apparently 
not  aware  of  Stanley's  observation,  he  inferred  (from  the  character 
of  the  symptoms  and  his  theory  that  the  posterior  columns  contain 
fibres  connecting  segments  of  the  cord  at  different  levels)  that  the 
posterior  columns  would  be  found  diseased;  and  he  verified  this 
inference  by  finding  in  two  cases  disease  of  these  columns.  The  credit 
of  tlie  discovery  of  the  disease  belongs,  if  to  anyone,  unquestionably 
to  Todd,  and  few  diseases  can  with  greater  truth  be  said  to  have  been 
"discovered."  Four  years  later  (in  1851)  Eomberg  described  the 
disease  and  the  lesion  in  the  posterior  o.olunms,  but  he  failed  to  exclude 
loss  of  power  from  the  syraptoms.  Russell  Eeynolds,  in  1855,  gave 
an  accurate  description  of  the  symptoms,  and,  in  attributing  the 
ataxy  to  lauscular  anaesthesia,  was  the  first  to  give  what  we  must  now 
regard  as  the  true  explanation  of  the  chief  symptom. f  A  series  of 
cases  was  described  by  G-ull  in  1856  and  1857.  Tiirck  fijrst  observed 
with  the  microscope  the  wasting  of  the  fibres  in  the  posterior  columns. 
Duchenne  in  1858-9  published  an  independent  and  very  able  analysis 
of  the  symptoms  of  the  disease,  and  gave  it  the  name  "locomotor 
ataxy."  He  obtained  for  it  (with  the  help  of  Trousseau's  "Lectures") 
the  recognition  that  previous  descriptions  had  failed  to  secure,  and 
achieved  such  a  degree  of  success  that  the  malady  is  called  in  France 
"  Duchenne's  disease."  If  any  name  is  attached  to  it,  that  of  Todd 
alone  can  be  right. 

Cattses. — The  disease  is  much  more  frequent,  in  this  country  at 
least,  in  urban  than  rural  populations.  Inherited  influence  is  to  be 
traced  only  in  a  small  proportion  of  the  cases,  perhaps  in  not  more 
than  10  per  cent.  It  is  usually  a  general  neurotic  heredity,  manifested 
by  such  diseases  as  insanity,  epilepsy,  and  other  degenerative  diseases 
of  the  nervous  system.  Instances  of  this  are — father  epileptic; 
father  insane ;  two  sisters  insane.  But  in  this  small  percentage  the 
influence  becomes  insignificant  in  the  presence  of  the  chief  cause,  to  be 
mentioned  presently,  since  in  many  instances  its  coincidence  must  be 
accidental.  Direct  inheritance  of  the  disease  is  extremely  rare  (the 
special  hereditary  fonn  being  always  excluded).  A  boy  with  distinct 
symptoms  and  optic  nerve  atrophy  was  the  son  of  a  man  who  pre- 
sented characteristic  indications  of  the  early  stage  of  tabes  (Eemak). 
In  most  instances  of  this  character  the  inheritance  is  not  direct,  being 
effected  by  means  of  syphilis.  The  inherited  disease  is  probably  the 
only  cause  of  the  juvenile  form  of  tabes. 

Males  suffer  far  more  frequently  than  f emaJes,  the  proportion  being 
about  ten  to  one,  and  this  seems  to  imply  some  proclivity  inherent  in 

•  *  Cyclopaedia  of  Anatomy  and  Physiolog-y,'  vol.  iii,  p.  721. 

+  Reynolds,  'Diagnosis  of  Diseases  of  the  Brain,  &c.,'  1855,  "Anaesthesia  Mas- 
cnlaris."  Certain  words  are  worth  quoting: — "It  appears  most  probable  that  the 
centripetal  tract  of  fibres  is  affected,  and  that  the  locality  of  lesion  is  very  variable  ** 
(p.  165). 


446  SPINAL    COED. 

the  male  sex.  A  like  preponderance  of  males  obtains  in  a  disease  tliat 
has  a  close  alliance  with  tabes — general  paralysis  of  the  insane.  It  is, 
however,  not  certain  that  the  sexual  difference  may  not  be  connected 
with  the  wider  dissemination  of  the  chief  cause — syphilis — among 
men,  and  with  some  peculiar  power  of  resistance  to  its  influence  on  the 
part  of  women.  The  middle  period  of  adult  life  is  that  in  which 
locomotor  ataxy  usually  commences.  No  less  than  half  the  cases  begin 
between  thirty  and  forty,  one  quarter  between  forty  and  fifty,  and 
rather  less  than  a  quarter  between  twenty  and  thirty.  It  rarely  begins 
after  fifty,  but  I  have  once  known  it  to  develop  at  sixty-six.  Under 
twenty  it  is  still  more  rare,  but  cases  are  met  with  as  early  as  ten,  and 
even  in  quite  young  children,  the  subjects  of  the  cause  next  described. 
Among  the  individual  causes,  one  overshadows  all  the  rest — ^the 
influence  of  syphilis.  A  very  large  proportion  of  the  sufferers  have 
had,  at  some  previous  time,  constitutional  syphilis,  either  distinct 
secondary  symptoms  or  an  indurated  sore.  The  proportion  is  almost 
as  large  in  the  upper  and  middle  classes  as  in  the  lower.  Of  175 
consecutive  cases  of  the  disease  in  men,  seen  in  private  practice  (in 
which  the  past  history  can  be  rehed  on  with  more  confidence  than  in 
hospital  patients),  no  less  than  114  of  the  cases,  or  69  per  cent.,  gave 
a  history  of  a  chancre  known  to  be  hard,  or  of  secondary  sym- 
ptoms ;  and  twenty-two  others  had  had  a  venereal  sore  of  unknown 
nature.  The  percentage  of  those  with  a  history  of  syphilis  or  a 
hard  chancre  is  77.  In  another  13  per  cent,  there  was  a  history  of 
gonorrhoea,  in  some  cases  of  repeated  attacks  ;  this  involves  exposure 
to  the  risk  of  syphilis.  Patients  with  unquestionable  secondary 
syphilis  are  often  ignorant  of  any  primary  sore.  When  a  disease  is  a 
distinct  sequel  of  syphilis  in  so  large  a  proportion  of  cases  as  69  per 
cent.,  we  are  justified  in  assuming  a  similar  relation  in  at  least  a 
majority  of  the  cases  in  which  infection  cannot  be  excluded.  Gonorrhoea 
emphasises  the  inabihty  to  exclude  syphilis,  but  in  no  less  than  3  per 
cent,  of  the  total  number  of  cases  was  there  no  exposure  to  the  risk  of 
contracting  syphilis  in  the  common  way.  In  the  lower  classes  the  pro- 
portion is  higher,  and  has  been  estimated  at  80  per  cent.,  or  even  more. 
The  ascertainable  facts  are  certainly  below  the  real  facts,  as  I  have 
pointed  out  elsewhere.*  When  a  deduction  is  made  for  possible 
accidental  coincidence,  there  remains  at  least  one  half  of  the  cases  in 
which  numerical  coincidence  must  depend  on  causal  relationship.  It 
is  probable,  indeed,  that,  taking  all  cases,  a  causal  proportion  of  three 
quarters  would  be  nearer  the  truth.  In  women  also  antecedent  syphilis 
can  often  be  traced,  although  rather  less  frequently  than  in  men.  At 
the  same  time  the  facts  are  even  more  diflicult  to  ascertain,  since  in 
married  women  syphilis  so  often  runs  a  latent  course.  But  in  the  cases 
of  tabes  that  succeed  syphilis  the  lesion  is  not  syphilitic  in  histological 
character ;  it  is  as  degenerative  as  in  the  cases  in  which  syphilis  can  be 
excluded,  and  it  is  not  influenced  by  the  treatment  for  syphilis.  Hence 
*  '  Lettsomiau  Lectures,'  1889. 


LOCOMOTOR    ATAXY.  447 

it  must  be  regarded  as  a  degenerative  sequel  of  syphilis  rather  than  as 
a  true  syphilitic  disease.  It  is  probably  the  influence  of  syphilis  that 
determines  the  greater  incidence  on  the  urban  than  on  the  rural  popula- 
tion, and  the  preponderance  of  cases  in  middle  life.  Inherited  syphilis  is 
also  capable  of  causing  the  disease,  and  is  to  be  traced  (as  far  as  1  have 
seen)  in  all  cases  in  children.  After  the  acquired  disease  the  interval 
between  primary  syphilis  and  the  first  symptoms  of  tabes  varies  from 
one  to  twenty  years.  It  is,  however,  rarely  less  than  three  years,  and 
in  most  cases  it  is  between  six  and  twelve  years.  In  rare  cases  the 
first  symptoms  occur  diuring  the  active  stage  of  syphilis.* 

It  is  probable,  however,  that  syphilis  is  not  the  only  cause  of  the 
disease.  In  a,  few  cases,  less  than  10  per  cent,  of  the  whole,  it  can  be 
excluded  with  confidence.  The  causes  operative  in  these  can  be 
traced  also  in  some  of  the  patients  who  present  a  history  of  syphilis, 
and  in  such  cases  the  causation  of  the  malady  is  probably  complex. 
Hitzig  and  Buzzard  have  suggested  that  a  toxin  having  a  similar 
degenerative  action  may  arise  from  soft  sores. 

One  of  these  causes,  which  can  sometimes  be  clearly  traced,  is 
injury,  such  as  involves  concussion  of  the  spine.  The  immediate 
results  of  the  injury,  whether  slight  or  grave,  transient  or  lasting,  are 
followed  by  the  symptoms  of  the  degenerative  malady.  In  one  case  a 
man  fell  from  a  height  on  to  the  deck  of  a  ship ;  transient  paraplegia 
resulted,  but  six  weeks  after  the  accident  the  man  presented  extrem«> 
ataxy,  with  good  power,  and  no  knee-jerk  (Arnold).  I  have  known 
the  symptoms  to  develop  gradually  a>  few  months  after  a  fall  from  a 
horse.  Exposure  to  cold  and  wet  has  occasionally  preceded  the  onse* 
so  directly  that  it  must  be  regarded  as,  at  least,  an  exciting  cause. 
There  is  a  history  of  syphilis  in  the  majority  of  cases  in  which  the 
manifestation  of  tabes  f oUows  some  immediate  cause.     In  other  casps 

*  Although  an  occasional  relation  to  syphilis  had  been  noted  by  several  preceding 
observers,  Fouvnier  was  the  first  {\n  1876)  to  assert  the  wide  extent  of  this  relation. 
His  statements  were  received  with  doubt  (because  syphilitic  patients  constituted  his 
field  for  observation),  but  they  were  confirmed  from  the  neurological  side  by  myself 
(♦  British  Med.  Journal,'  March  1, 1879)  and  Erb  ('  Arch,  f .  klin.  Med./  July,  1879)  ; 
while  abundant  corroboration  has  been  since  afforded.  Many  who  at  first  doubted 
have  been  convinced  by  fresh  observations.  Facts  collected  without  referen«".e  to 
any  point  at  issue  are  generally  worthless,  and  freshly  collected  facts  have  thrown 
a  new  light  on  the  subject.  The  proportion  of  cases  with  previous  syphilis  neces- 
sarily varies  according  to  the  absolute  frequency  of  syphilis.  The  difierence  in  the 
character  of  the  lesion  from  that  of  changes  known  to  be  syphilitic  was  urged  as  an 
objection,  but  such  considerations  are  theoretical,  and  must  yield  to  facts  ;  in-.tead 
of  denying  that  this  or  that  lesion  can  be  produced  by  a  given  cause,  we  may  have 
to  widen  our  view  of  the  operation  of  that  cause.  Other  degenerations  of  the 
nervous  system  seem  also  to  have  a  relation  to  syphilis,  as  I  pointed  out  some  years 
ago  ('Lancet,'  Jan.  15,  1881).  As  will  be  shown  in  the  section  on  Pathology, 
recent  discoveries  lessen  very  much  the  difficulty  of  comprehending  the  relation- 
ship by  demonstrating  the  profound  influence  on  the  nervous  system  of  chemical 
toxins  generated  by  the  organisms  which  we  have  learned  to  discern  as  th^ 
materies  morhi  of  specific  diseases. 


448  SPINAL   COllD. 

the  disease  has  succeeded  excessive  fatigue  and  over-exertion,  and  also 
certain  acute  diseases,  especially  (it  is  said)  acute  rheumatism  and 
typhoid  fever ;  it  perhaps  follows  diphtheria  in  rare  cases,  but  most 
cases  have  been  examples  of  the  ataxic  form  of  diphtheritic  paralysis, 
ultimately  passing  away.  Alcoholic  excess  has  been  noted  in  some 
instances,  but  most  recorded  instances  have  been  due  to  peripheral 
neui'itis,  involving  chiefly  the  afferent  muscle-nerves.  Sexual  excess 
has  been  supposed  by  some  to  be  a  cause ;  but  its  influence  can  rarely 
be  detected,  and  its  significance  is  uncertain,  since  sexual  excitement  is 
undoubtedly  sometimes  an  early  symptom  of  the  disease. 

Secondary  Tabes. — Symptoms  of  locomotor  ataxy  sometimes  succeed 
other  diseases  of  the  spinal  cord,  and  such  sequence  is  especially 
common  in  syphilitic  subjects.  Myelitis  and  syphilitic  gummata 
may  be  thus  succeeded  by  tabes.  An  officer  in  India,  who  had  had 
syphilis,  having  suffered  for  a  day  or  two  from  pains  in  the  back,  took 
a  bath  in  snow  water,  and  in  a  few  days  his  legs  were  absolutely 
powerless.  He  gradually  recovered  power,  but  could  not  co-ordinate 
the  movement:  as  power  returned,  lightning  pains  came  on,  and  a 
year  afterwards  he  presented  the  typical  condition  of  locomotor  ataxy. 
Again,  a  man,  twelve  years  after  syphilis,  had  a  severe  fall,  followed 
by  gradual  loss  of  power,  so  that  at  the  end  of  three  weeks  he  could 
scarcely  stand.  The  legs  remained  weak  for  a  month,  and  then  im- 
proved, but  ataxy  came  on ;  three  months  later,  power  was  good,  inco- 
ordination extreme.  In  many  cases,  however,  the  ultimate  condition 
is  one  of  combined  weakness  and  ataxy. 

Symptoms. — A  typical  case  of  developed  tabes  j)resents  certain 
motor,  sensory,  and  reflex  symptoms.  There  is  inco-ordination  of 
movement  of  the  legs,  sometimes  of  the  arms  also,  without  loss  of 
power  or  muscular  wasting.  There  are  pains  in  the  affected  parts, 
especially  sharp  momentary  "  lightning  "  pains  ;  there  is  some  loss  of 
sensation ;  there  is  often  loss  or  diminution  of  reflex  action  from  the 
skin,  and  almost  always  entire  loss  of  the  myotatic  irritability  that  is 
revealed  by  the  so-caUed  "  tendon-reflexes,"  and  especially  by  the  knee- 
jerk;  there  may  be  retention  or  incontinence  of  urine,  constipation, 
and  often  loss  of  sexual  power.  Of  this  group  of  symptoms,  two 
usually  precede  the  others — the  pains,  and  the  loss  of  the  knee-jerk. 
These  may  exist  alone,  even  for  years,  before  inco-ordination  comes  on. 
Thus  the  symptoms  are  far  wider  in  range  than  the  name  "  ataxy " 
suggests ;  and  while  inco-ordination,  if  it  exists,  is  the  most  obtrusive 
objective  symptom,  it  may  never  be  developed.  Hence  physicians 
have  extensively  fallen  back  on  the  older  term  "tabes  dorsahs."  But 
recent  expansion  of  our  knowledge,  especially  the  discovery  of  the 
extent  to  which  the  symptoms  may  depend  on  disease  of  the  peri- 
pheral nerves,  shows  that  even  the  qualifying  "  dorsalis  '*  narrows  the 
term  unduly,  and  "  peripheral  neuro-tabes  "  has  been  added  to  the 
terminology  of  the  disease. 

Besides  the  symptoms  above  enumerated,  others  are  occasionallj 


LOCOMOTOR    ATAXY.  449 

present.  Of  these  tlie  most  important  are  atrophy  of  the  optic  nerve 
(and  occasionally  of  other  cranial  nerves) ;  trophic  changes  in  the  sl^in, 
the  bones,  and  the  joints ;  peculiar  paroxysmal  visceral  disturbances 
and  occasionally  motor  paralyses  and  muscular  -wasting.  The  com- 
binations of  symptoms  present  in  different  cases  vary  much.  It  will 
be  convenient  to  consider  first  the  individual  symptoms  in  their 
various  degrees,  and  then  their  grouping  and  sequence. 

Motor  Symptoms. — The  characteristic  inco-ordination  of  movement 
develops  gradually.  It  is  usually  increased  (as  Eomberg  pointed  out ) 
by  closui'e  of  the  eyes,  and  at  first  may  only  exist  when  the  guiding 
influence  of  vision  is  thus  withdrawn.  Before  it  causes  ataxy  of 
movement  it  may  render  difficult  the  maintenance  of  equilibrium  when 
the  base  of  support  is  narrowed  by  the  feet  being  placed  close  together, 
toes  and  heels ;  if  then  the  eyes  are  closed  the  patient  sways,  and 
tends  to  fall.  The  effect  of  closure  of  the  eyes  is  greatest  when 
sensation  in  the  soles  of  the  feet  is  defective,  but  does  not  depend  on 
this  defect ;  it  may  be  marked  when  sensation  on  the  soles  of  the  feet 
is  perfect.  The  early  defect  in  co-ordination  may  be  discovered  by 
the  patient  when  he  walks  in  the  dark  or  backwards,  or,  not  un- 
commonly, when  he  shuts  his  eyes  in  the  process  of  washing  the  face. 
In  a  further  degree  of  inco-ordination  there  is  inability  to  stand  with 
the  feet  together,  even  when  the  eyes  are  open,  and  the  patient  is  only 
steady  when  the  feet  are  wide  apart.  If  the  feet  are  bare,  the  diffi- 
culty is  greater,  because  muscular  action  has  to  replace  the  rigid  base 
of  the  boot.  The  irregidar  contraction  of  the  muscles  is  shown  by  the 
conspicuous  movement  of  the  tendons  on  the  back  of  the  feet.  The 
patient  may  oscillate  from  toes  to  heels  before  he  comes  to  rest.  As 
the  defect  progresses,  uncertainty  is  felt  in  walking  even  with  full 
visual  guidance,  especially  on  uneven  ground,  or  on  a  very  smooth 
surface.  A  slight  visible  alteration  in  gait  is  then  appreciable,  the 
feet  are  not  placed  on  the  ground  quite  as  in  health,  or  there  is  distinct 
difficulty  in  maintaining  equilibrium  when  the  patient  turns  quickly, 
and  he  has  to  put  a  foot  down  suddenly  to  keep  from  falling.  As  the 
inco-ordination  increases,  the  change  in  gait  becomes  greater,  but 
varies  much  in  its  precise  characters,  according  to  the  muscles  that 
are  most  affected.  Often  the  feet  are  raised  too  high,  thrown  for- 
wards too  far,  brought  down  too  suddenly,  and  the  whole  sole  comes 
in  contact  with  the  ground  at  once.  Often  the  foot  becomes  inverted 
when  it  touches  the  ground.  Efforts  to  correct  error  in  movement 
have  themselves  to  be  corrected.  In  other  cases  the  defect  in  main- 
taining equilibrium  is  greater  than  the  actual  disorder  of  movement  of 
the  legs,  and  the  patient  sways  about  in  the  manner  of  one  who  has 
cerebellar  disease ;  this  probably  depends  on  a  preponderant  affection 
of  the  muscles  of  the  hip-joint. 

As  the  defect  progresses,  the  patient  is  only  able  to  walk  by  steady- 
ing himself  with  a  stick,  or  by  taking  hold  of  the  arm  of  another  person 
or  of  adjacent  objects.     At  first  a  very  slight  degree  of  this  help  is 

VOL.  I.  29 


450  SPINAL    CORD. 

sufficient ;  guidance  rather  tlian  support  is  needed.  Afterwards, 
however,  considerable  support  is  necessary,  and  ultimately  the  patient 
may  be  unable  to  stand  even  with  help.  "When  he  attempts  to  rise, 
the  legs  move  hurriedly  forwards  and  backwards,  and  if  the  upright 
posture  is  at  last  achieved,  the  legs  slip  forward,  and  only  strong 
support  saves  the  patient  from  a  fall.  The  ataxy  is  manifest  also  in 
other  movements  of  the  legs.  If  the  patient,  when  lying,  tries  to 
touch  an  object  with  his  foot,  the  leg  is  moved  irregularly,  goes  beyond 
the  place,  and  then  is  brought  too  far  back,  and  only  at  last  does  it 
come  in  contact  with  the  object,  often  with  unintended  force.  This 
inco-ordination,  like  that  in  standing,  is  much  greater  if  the  eyes  are 
closed. 

The  arms  may  present  similar  inco-ordination,  although  they  often 
escape,  even  when  the  affection  of  the  legs  is  extreme ;  very  rarely  the 
arms  suffer  before  the  legs  (brachial  tabes).  In  one  such  case  there 
was  loss  of  sensation  to  touch,  not  only  in  the  arms,  but  on  the  trunk 
between  the  epigastric  level  and  the  distribution  of  the  cervical  plexus. 
The  commencing  defect  is  revealed  by  delicate  movements,  such  as 
writing.  When  slight,  it  may  be  conspicuous  if  the  patient  tries,  with 
closed  eyes,  to  touch  some  object,  such  as  his  own  nose,  or,  having 
abducted  his  arms,  tries  to  bring  the  forefingers  together.  As  it 
increases,  all  movements  become  irregular ;  it  is  impossible  for  the 
patient  to  button  his  coat,  or  to  pick  up  a  small  object  from  the  table ; 
the  fingers  twist  about  in  the  attempt.  The  grasp  is  not  sustained ; 
first  one  finger  is  felt  to  relax  and  then  another.  If  the  patient 
attempts  to  hold  out  his  hand  in  a  fixed  posture,  it  is  seen  that  the 
same  irregularity  obtains ;  instead  of  a  uniform  balanced  contraction 
the  muscles  contract  and  relax  involuntarily,  and  slow  unintended 
movements  of  the  fingers  result,  sometimes  closely  resembling  those  of 
athetosis.  The  same  spontaneous  movements  may  also  be  observed  in 
the  legs.  They  cease  at  once  when  the  muscular  effort  is  relinquished. 
Occasionally  the  muscles  of  the  trunk  present  inco-ordination.  Thus 
one  patient  could  sit  steadily  on  a  chair  when  his  eyes  were  open,  but 
if  he  closed  them  would  at  once  fall  off.  The  movements  of  the  head, 
face,  tongue,  and  eyes  escape  the  characteristic  derangement. 

Even  with  extreme  inco-ordination,  the  power  of  the  muscles  may  be 
unimpaired.  Occasionally  some  group  of  muscles,  as  the  flexors  of  the 
ankle,  become  weak  for  a  little  time  and  then  strong  again,  just  as  may 
the  eyeball  muscles,  as  we  shall  presently  see.  In  some  cases  motor 
power  in  the  limbs  remains  unimpaired  to  the  end ;  more  often,  when 
the  ataxy  has  become  great,  some  muscular  weakness  supervenes,  with 
or  without  wasting  of  the  weak  muscles  There  is  a  distinct  group  of 
cases  in  which  weakness  and  ataxy  come  on  together,  but  these  are 
considered  separately  ("  Ataxic  Paraplegia  ") . 

Sensory  symjdoms  are  prominent  in  most  cases, — subjective  sensa- 
tions, especially  of  pain,  and  loss  of  sensibiKty.  Spontaneous  pains 
are  present  in  some  degree  in  nine  tenths  of  the  cases  of  tabes.     The 


LOCOMOTOR  ATAXY.  451 

most  frequent  and  characteristic  are  tlie  sudden  and  lancinating  pains 
called  "  lightning  pains."  They  occur  cMefly  in  the  legs,  but  may  be  felt 
in  the  trunk,  arms,  and  even  in  the  head  and  face.  They  are  usually 
paroxysmal,  and  apt  to  come  on  at  night ;  attacks  of  such  pains  last 
for  some  hours  or  for  a  day  or  two,  varying  in  seat,  but  often  felt  in 
the  same  part  throughout  an  attack,  or  through  many  attacks. 
Sometimes  they  are  felt  in  a  limited  area  of  the  skin ;  at  others  they 
seem  more  deeply  seated ;  sometimes  they  dart  down  the  limb.  They 
may  correspond  to  a  nerve-trunk  (especially  the  sciatic),  but  more 
frequently  have  no  relation  to  the  nerves.  They  may  be  felt  near 
joints,  but  ai-e  seldom  referred  to  them.  Although  the  pains  are 
usually  acute  and  "  stabbing,"  they  are  sometimes  of  different  cha- 
racter. They  may  be  compared  to  bad  toothache,  even  when  lasting 
only  a  second  or  two.  In  one  patient  the  sensation  was  as  if  both 
legs  were  for  a  moment  on  fire.  The  pains  in  the  trunk  sometimes 
correspond  in  seat  to  a  zone  of  hypersesthesia,  especially  when  there  is 
a  marked  "  girdle  pain."  When  the  pains  are  referred  to  the  skin, 
this  often  becomes  tender  after  the  pains  have  been  felt  for  a  time ;  the 
contact  of  the  bedclothes  cannot  be  borne,  and  even  a  breath  of  air  can 
scarcely  be  endured.  Curiously,  this  may  be  the  case  when  there  is 
impaired  sensibility  to  pain,  but  not  to  touch.  When  the  pain  is  iu 
the  face  it  may  closely  simulate  trigeminal  "  tic ;"  only  it  is  on  both 
Bides,  and  such  symmetrical  facial  neuralgia  is  very  suggestive  of  a 
tabetic  origin. 

When  pains  continue  at  one  spot  for  some  days,  vascular  or  trophic 
changes  may  occur  there, — ecchymoses  have  been  observed,  and  I  have 
known  the  growth  of  the  hair  to  be  changed  where  pains  had  been  felt 
in  the  scalp ;  each  hair  bent  and  broke  off  near  the  skin,  over  an  area 
the  size  of  half  a  crown;  after  the  pains  ceased  the  growth  of  the 
hair  became  normal.  A  sharp  pain  may  be  accompanied  by  a  sudden 
reflex  spasmodic  movement  of  the  legs,  or  by  inhibitory  weakness. 
Thus,  in  one  case,  a  sudden  pain  would  often  make  the  patient  fall  on 
his  knees.    The  pains  may  be  so  severe  as  to  prevent  sleep. 

Other  kinds  of  severe  pain,  less  brief  than  the  lightning  pains,  occur 
in  some  cases,  and  are  described  by  various  epithets,  as  "  burning," 
''tearing,"  "gnawing."  Burning  pain  in  the  toes  distressed  one 
patient.  Much  more  common,  and  occurring  with  other  pains,  are 
slighter  dull  pains  like  those  of  rheumatism,  for  which  they  are  often 
mistaken,  an  error  that  is  facilitated  by  the  circumstance  that  any  of 
the  pains  of  tabes  may  be  influenced  by  weather,  being  especially  in- 
creased by  damp  cold.  Indeed,  this  occurrence  on  any  sudden  change 
in  weather  is  often  a  marked  featxire.  It  is  not  uncommon  for  a 
patient  to  describe  them  as  "  equivalent "  to  a  barometer.  They  may 
also  be  excited  by  indigestion.  Dull  pains  sometimes  precede  the 
sharp  pains,  occasionally  for  years.  A  painful  sense  of  constriction 
"  girdle-pain,"  is  also  common,  and  may  be  felt  aroiond  the  trunk  or  in 
the  legs,  groins,  &c. ;  it  is  sometimes  referred  to  a  considerable  area — 


452  SPINAL   CORD. 

the  patient  may  feel  as  if  tlie  whole  trunk  were  tightly  enveloped  in  a 
cuirass  of  metal.  Darting  pains  also  may  be  felt  in  the  trunk  and  not 
in  the  legs.  Such  a  sense  of  constriction  is  sometimes  referred  to  the 
legs,  especially  to  the  knees,  but  sometimes  to  the  feet.  One  patient 
described  this  sensation  as  if  an  iron  band  were  around  each  foot.  In 
the  arms  and  hands  the  pains  are  usually  sHght  in  degree,  and  seldom 
cause  real  distress ;  they  are  often  felt  chiefly  in  the  ulnar  region. 
Visceral  pains  may  also  occur,  usually  paroxysmal,  and  referred  to 
the  stomach,  bladder,  rectum,  &c. 

Pains  are  not  only  the  most  common,  they  are  often  the  earliest 
indication  of  the  disease,  and  may  be  the  only  subjectire  symptoms 
in  slight  and  stationary  cases.  They  bear  no  proportion  to  the  other 
symptoms,  and  are  sometimes  trifling — occasionally  altogether  absent. 
They  seem  sometimes  to  occur  alone;  I  have  seen  several  patients 
■who  suffered  lightning  pains,  characteristic  in  features,  course,  and 
general  associations,  both  with  and  without  the  pupil  symptoms 
common  in  tabes,  but  with  no  loss  of  the  knee-jerk.  The  pains 
are  then  usually  superficial,  and  the  cases  may  be  termed  "  cuta- 
neous neuro-tabes."  Ataxy  may  supervene,  with  loss  of  the  knee- 
jerk. 

Sensations  other  than  pain  are  also  common.  They  are  various  in 
character,  described  as  "  tinghng,"  "pins  and  needles,"  "creeping," 
and  very  often  as  if  the  skin,  especially  of  the  soles,  were  covered  by 
some  soft  substance.  Sensations  of  cold  or  of  heat  are  also  common ; 
the  feet  may  feel  as  if  the  legs  are  always  in  cold  water ;  a  sensation 
of  cold  about  the  testicles  is  occasionally  complained  of.  Similar 
cutaneous  sensations  are  also  common  in  the  hands.  They  are  espe- 
cially common  in  the  distribution  of  the  ulnar  nerve.  Here  also 
there  may  be  impaired  sensibility,  especially  for  pain  and  temperature, 
when  the  sensory  condition  elsewhere  in  the  arm  is  normal.  The 
Tilnar  nerve  has  also  been  found  insensitive  to  pressure  (Biernacki). 
Increased  sensitiveness  to  pain  may  also  exist,  especially  on  the  soles 
of  the  feet,  and  often  accompanies  lessened  sensibility  to  touch. 

A  diminution  of  sensibility  is  also  present  in  most  cases  of  the 
developed  disease.  It  may  involve  all  forms  of  sensibility,  but  seldom 
equally;  some  forms  may  be  impaired  and  others  normal.  Either 
pain  or  touch  may  be  affected  alone ;  the  most  frequent  change  is  loss 
of  sensitiveness  to  pain.  Delay  of  the  perception  of  painful  stimuli  is 
a  common  early  symptom,  as  will  presently  be  described.  A  firm 
touch  (i.  e.  slight  pressure)  may  be  felt  when  a  slight  touch  is  unper- 
ceived.  When  a  touch  is  felt,  the  power  of  locaHsing  it  may  be  im- 
paired. Temperature  sensations  are  rarely  affected  without  other 
forms  of  sensibility,  but  they  are  often  impaired  with  that  of  paia 
when  tactile  sensibility  is  normal.  On  the  other  hand,  there  may 
be  extensive  loss  to  pain  without  any  defect  of  the  temperature  sense. 
A  prick  may  then  cause  only  a  sensation  of  heat  or  burning,  and 
extreme  degrees  of  heat  or  cold  cause  pain  less  readily  than  normal 


LOCOMOTOR    ATAXY.  453 

The  temperature  loss  may  be  partial, — either  cold  or  heat  may  be 
•un perceived  wbile  tbe  other  is  recognised.  When  touch  is  slightly 
impaired,  there  may  be  an  inability  to  discriminate  differences  in 
the  degree  of  pressure  on  the  skin. 

Accompanying  the  diminution  or  increase  of  sensitiveness  there  are 
sometimes  curious  changes  in  the  sensation  produced.  One  of  these 
is  a  delay  in  the  perception  of  pain,  which  may  amount  to  several 
seconds.  The  greatest  delay  I  have  met  with  amounted  to  seven 
seconds,  but  one  of  fifteen  seconds  has  been  described  (Eulenberg). 
The  contact  of  a  point  may  be  felt  at  once,  the  pain  only  after  a 
considerable  interval.  When  felt  it  may  be  excessive  in  degree. 
Often  there  is  an  after-pain,  lasting  sometimes  for  a  quarter  or  haK  a 
minute  or  longer,  and  the  maximum  sensation  may  not  be  attained 
until  several  seconds  after  the  pain  is  first  felt.  Thus,  in  a  case  in 
which  there  was  a  delay  of  seven  seconds,  the  maximum  sensation  was 
only  felt  twenty-five  seconds  after  the  prick  (Obersteiner).  Often 
there  is  a  second  or  third  maximum,  a  sort  of  rhythmical  recurrence 
of  sensation,  quick  or  deliberate.  A  delay  greater  than  normal  may 
also  attend  the  sensation  of  heat,  but  this  is  less  easy  to  recognise  on 
account  of  the  time  normally  required  to  raise  the  temperature  of  the 
skin.  The  localising  power  is  sometimes  strangely  perverted.  A 
touch  on  the  toe  may  be  referred  to  the  back  of  the  foot  even  when 
there  is  no  ataxy.  A  touch  or  prick  on  one  leg  may  be  referred  to  the 
other,  sometimes  with  singular  uniformity  in  position  ("  allocheiria," 
p.  14).  A  prick  in  one  spot  may  be  felt  in  many  places  ("  polyaes- 
thesia")  on  the  same,  or  on  both  legs.  There  may  also  be  impair- 
ment on  the  face  corresponding  roughly  to  divisions  of  the  fifth  nerve. 
Occasionally  a  patient  feels  as  if  the  face  were  covered  by  a  mask, 
stretching  and  compressing  the  skin,  a  symptom  named  by  Charcot 
"  Hutchinson's  mask."  The  reaction  of  the  sensoiy  nerves  to  elec- 
tricity may  be  changed  in  the  same  way  as  that  of  the  motor  nerves 
when  they  are  degenerated ;  instead  of  the  earliest  sensation,  on  closure 
of  the  circuit,  occurring  at  the  kathode,  it  occurs  at  the  anode  (Men- 
delssohn) . 

Impairment  of  sensation  is  a  very  variable  symptom.  It  may  be 
absent  even  when  the  spontaneous  pains  are  severe.  It  is  most  fre- 
quent, and  usually  greatest,  in  the  lower  part  of  the  legs  and  feet. 
It  may  even  be  limited  to  the  soles,  and  usually  then  ceases  at  the 
outer  sharp  edge  of  the  foot,  but  extends  for  an  inch  or  so  up  the 
inner  side.  Touch  or  pain  may  be  lost  on  the  sole  only,  and  even, 
strange  to  say,  the  form  of  sensibility  that  is  lost  on  the  sole  may 
alone  persist  in  the  legs.  Although  the  sole  is  usually  most  affected, 
sensation  may  be  perfect  on  the  sole  when  lost  elsewhere.  One  leg  is 
often  more  affected  than  the  other.  The  loss  may  extend  to  the  trunk, 
and  over  part  or  the  whole  of  it.  Defective  sensibility  to  touch  is 
sometimes  met  with  around  the  anus,  perinseum,  scrotum,  &c.,  and 
sexual  loss  is  said  to  be  usually  issociated  with  loss  of  sensibility  Ln 


454  SPINAL  CORD. 

the  glans  penis.*  The  characteristic  pain  which  pressure  on  the  tes- 
ticles produces  is  frequently  absent.  In  the  upper  limbs  the  impair^ 
inent  of  sensation  is  usually  greatest  on  the  hands ;  it  often  commences 
on  the  palmar  aspect  of  the  fingers,  and  may  be  limited  to  this 
aspect  of  the  hands,  just  as  it  is  to  the  soles  of  the  feet.  Sensation 
may  be  lost  on  the  trunk  and  not  on  the  limbs — an  important  fact, 
because  the  anaesthesia  may  then  readily  be  overlooked.  The  impair- 
ment on  the  trunk  frequently  ceases  when  the  level  of  the  third  rib  is 
reached.  On  the  head,  loss  of  sensation  is  rare  and  usually  partial. 
But  almost  universal  defect  of  sensitiveness  to  pain  over  the  whole  head 
is  sometimes  met  with. 

The  loss  of  sensibility  is  not  confined  to  the  skin,  but  affects  the 
deeper  tissues  also  in  many  cases.  The  contraction  of  the  muscles 
on  electrical  stimulation  may  be  unfelt,  and  the  muscles  may  be  less 
sensitive  than  normal  to  pressure  or  forcible  extension.  It  is  only 
from  intelligent  patients  that  this  can  be  distinctly  ascertained ;  but 
some  degree  of  defect  is  probably  an  early  change.  In  a  case  in  which 
the  inco- ordination  was  much  greater  in  one  leg  than  the  other,  in  the 
more  ataxic  leg  the  muscles  were  insensitive  to  traction,  which  excited 
a  definite  sensation  on  the  other  side.  The  muscles  may  indeed  be 
quite  insensitive  when  cutaneous  sensibility  is  little  or  not  at  all 
impaired.  It  is  probable  that  the  sensibility  of  the  joints,  fibrous 
tissues,  and  tendons  is  also  involved.  The  sense  of  posture  may  be  lost 
when  cutaneous  sensibility  is  intact.  The  anaesthesia  may  extend  to  the 
viscera  when  the  trunk  is  involved.  In  consequence  of  the  loss  of 
sensation,  grave  injuries  to  the  limbs,  such  as  burns,  may  be  unper- 
ceived,  and  visceral  disease  may  be  unattended  with  the  customary 
pain.     Pleurisy,  for  instance,  may  be  absolutely  painless. 

But  impairment  of  sensation  is  not  invariable.  There  may  be  no 
loss  even  in  patients  who  present  considerable  inco- ordination,  and  in 
those  who  suffer  severe  pains,  as  well  as  in  those  who  have  had  none. 
But  it  is  very  rare  to  have  no  sensory  defect  when  inco- ordination  ia 
considerable. 

Reflex  action  from  the  skin  is  usually  impaired  in  proportion  to  the 
loss  of  cutaneous  sensibility,  and  especially  to  the  loss  of  tactile  sensi- 
biUfcy.  It  may  be  normal  when  pain  is  unfelt.  The  plantar  reflex 
is  most  frequently  impaired,  and  the  progress  upwards  of  the  disease 
may  be  attended  by  a  progressive  loss  of  the  gluteal,  cremasteric,  and 
abdominal  reflexes.  On  account  of  the  variations  met  with  in  health, 
it  is  uncertain  whether  reflex  action  is  ever  lessened  when  there  is  no 
impairment  of  sensation.  On  the  other  hand,  in  the  early  stage  of 
the  disease  there  is  sometimes  a  considerable  and  even  extreme  excess 
of  all  cutaneous  reflex  action. 

Loss  of  sexual  power  is  an  exceedingly  common  and  often  an  early 
symptom  of  the  disease,  and  may  precede  any  impairment  of  sensory 
or  reflex  functions.     As  has  already  been  stated,  the  loss  is  said  as 
*  Brown, '  Lancet,'  1898. 


LUCOMOTOE    ATAXY.  455 

a  rule  to  "be  accompanied  hj  loss  of  sensation  in  the  glans  penis.  Tet 
in  snch.  cases  nocturnal  emissions  may  occur,  sometimes  very  frequently. 
On  the  other  hand,  exceptions  are  met  with  in  which  sexual  power 
persists  to  a  late  period  of  the  disease,  even  until  after  the  patient  has 
lost  the  ability  to  walk  alone. 

If  the  condition  of  cutaneous  reflex  action  is  somewhat  inconstant, 
this  is  not  the  case  with  the  reflex  process  on  which  the  myotatic 
irritability  depends.  A  loss  of  the  knee-jerk,  as  Westphal  first 
pointed  out,  is  one  of  the  earliest  and  most  constant  symptoms  of  tabes. 
With  it  disappear  also  all  other  indications  of  this  irritability.  In 
cases  with  great  excess  of  cutaneous  reflex  action  the  knee-jerk  is  lost 
as  in  other  cases,  and  the  contrast  between  the  two  forms  of  reflex 
action  is  very  striking.  It  is  exceedingly  rare  for  the  knee-jerk  to  be 
obtained  in  any  case  of  true  tabes,  but  an  early  case  is  sometimes  met 
with  in  which  it  is  not  lost,  although  diminished,  and  commonly 
unequal  on  the  two  sides.  In  one  or  two  cases  I  have  found  it  slight 
on  one  side  and  lost  on  the  other  when  distinct  ataxy  was  present,  and 
have  observed  its  subsequent  disappearance.*  In  one  ca^e,  in  which 
the  jerk  was  lost  in  one  leg  only,  lightning  pains  were  confined  to  this 
leg.  Yery  rarely  the  knee-jerk  persists  and  is  equal,  although  there 
are  characteristic  pains,  loss  of  the  iris  reflex,  and  a  history  of  syphilis. 
The  loss  often  precedes  for  many  years  the  development  of  inco- 
ordination. It  is  important  to  remember  that  a  true  reflex  action  may 
simulate  the  knee-jerk  when  it  is  lost.  It  is  distinguished  by  the  con- 
spicuous interval  between  the  blow  and  the  movement,  by  the  wide 
range  of  this,  and  by  the  fact  that  a  similar  effect  can  be  produced  by 
a  prick  on  the  skin  over  the  patellar  tendon. 

Eye  Symptoms. — The  functions  of  the  internal  muscles  of  the  eye  are 
often  affected  in  tabes.  In  more  than  five  sixths  of  the  cases  f  the 
reflex  action  of  the  iris  to  light  is  lost ;  rarely  the  action  remains  perfect 
throughout.  In  most  cases  of  reflex  loss  the  contraction  m  iccommo- 
dation  is  preserved,  as  first  noted  by  Argyll  Eobertson,  Often  also  (as 
Erb  has  shown)  the  dilatation  that  occurs  on  painful  stimulation  of 
the  skin  of  the  neck,  &c.,  can  no  longer  be  produced.  Sometimes  the 
ciliary  muscle  is  also  paralysed,  causing  loss  of  accommodation.  Yery 
rarely  there  is  loss  of  accommodation  and  of  the  associated  contraction 
of  the  iris,  without  loss  of  the  light-reflex.  When  reflex  contraction  is 
not  lost  it  may  not  be  maintained ;  the  pupil  again  enlarges  ;  an 
actual  inverted  reaction  to  light  and  darkness  has  been  observed. J 
The  pupils  are  often  very  small  ("  spinal  myosis  "),  especially  when 
there  is  loss  of  the  reflex  dilatation  from  the  skin,  a  loss  which  seems 
to  be  accompanied  by  atony  of  the  radiating  fibres  supplied  by  the 
sympathetic.     The  pupils  are  not  always  small ;   they  may  be  of  a 

*  Its  return  on  the  paralysed  side  in  a  tabetic  patient  who  became  liemiplegic, 
has  bpen  recorded  (Hughlings  Jackson  and  James  Taylor,  '  Brit.  Med.  Journ.,' 
vol.  i,  1894). 

f  Berger  says  97  per  cent. 

J  L'Abundo, '  La  Psych  iatria,'  1889,  vii,  p.  286. 


456 


SPINAL    CORD. 


medium  size  or  still  larger,  and  tlien  I  have  usually  found  tliat  the 
sldn-reflex  can  be  obtained,  although  the  light-reflex  cannot.  They 
are  often  not  perfectly  circular,  and  are  frequently  unequal.  Both  eyes 
are  usually  affected  in  the  same  manner  ;  occasionally  the  reflex  action 
is  lost  in  one  and  only  lessened  in  the  other ;  this  is  true  also  of 
accommodation  ;  indeed,  almost  every  possible  condition  is  occasionally 
met  with.  On  the  other  hand,  the  intra-ocular  muscles  may  be 
unaffected  even  in  advanced  cases.  According  to  Berger  the  ocular 
te'"  '  ui  IS  often  lessened. 

Paralysis  of  the  external  ocular  muscles  is  also  common  in  tabes, 
and  occurs  in  several  forms.      (1)  Transient  weakness  lasting  a  few 


Fia.  119. — ^Unilateral  tabetic  ptosis, 


Fig.  120. — Double  tabetic  ptosis. 


days  or  weeks,  and  then  passing  away.  (2)  Permanent  paralysis, 
complete  or  incomplete,  of  a  single  nerve  or  part  of  a  nerve.  Either 
form  may  occur  at  any  stage,  but  the  first  is  most  common  in  the 
early,  and  the  second  in  the  later  stages  of  the  disease.  The  transient 
palsy  renders  diplopia  a  common  symptom  in  the  early  period ;  any 
muscle  may  be  affected,  but  the  external  rectus  is  that  which  most 
frequently  suffers.  Transient  ptosis  may  precede  an  almost  complete 
external  ophthalmoplegia.  The  persistent  palsy  may  affect  one  or  more 
muscles ;  often  the  levator  is  involved,  causing  what  has  been  termed 
"tabetic  ptosis  "  (Figs.  119  and  120) .  Sometimes  the  whole  third  nerve 
is  paralysed.  (3)  There  may  be  a  combined  palsy,  suggesting  a  central 

*  Fi  om  a  Salpetriere  photograph,  for  which  I  am  indebted  to  M.  Charcot. 


LOCOMOTOE   ATAXY.  457 

origin — as,  for  instance,  loss  of  tlie  movement  of  convergence  asso- 
ciated with  loss  of  accommodation,  alttougli  tlie  internal  recti  act  in 
tlie  movement  towards  one  side.  (4)  Many  or  all  the  muscles  of  both 
eyes  may  gradually  become  paralysed — "  progressive  ophthalmoplegia," 
internal  or  external,  a  condition  that  is  more  fully  described  in  the 
account  of  ocular  palsies  in  Yol.  II. 

Atrophy  of  the  optic  nerve  is  the  most  serious  ocular  complication 
of  tabes.  Its  frequency  is  difficult  to  ascertain,  but  is  certainly  less 
than  is  often  supposed ;  it  does  not  occur  in  more  than  one  case  in 
ten.  Atrophy  is  generally  an  early  symj^tom,  usually  commencing 
before  inco-ordination  is  developed,  and  in  a  large  number  of  cases  ataxy 
does  not  supervene  ;  if  it  does,  the  arms  are  frequently  affected  before 
the  legs.  There  seems  a  tendency  for  the  spinal  malady  to  become 
stationary  when  the  optic  nerve  suffers.  The  disc  becomes  pale,  and 
its  substance  shrinks,  so  that  recession  begins  at  its  edge  instead  of  at 
the  central  "  cup,"  as  may  readily  be  perceived  by  the  course  of  the 
vessels.  The  failure  of  sight  usually  commences  with  a  peripheral 
limitation  of  the  field  and  loss  of  colour  vision,  but  sometimes  central 
acuity  fails  early.  When  this  remains  good,  the  peripheral  loss  may 
reach  a  considerable  degree  before  it  is  suspected  by  the  patient. 
Earely  there  is  a  symmetrical  sectorial  defect  in  the  fields.  The 
visible  pallor  of  the  optic  disc  is  not  always  proportioned  to  the 
failure  of  sight ;  the  change  in  tint  depends  on  wasting  of  the  capil- 
laries, and  this  does  not  always  correspond  to  the  wasting  of  the  nerve- 
fibres.  The  ultimate  tint  is  grey,  or  a  whitish  grey,  especially  as  seen 
by  the  direct  method.  When  the  disc  was  very  vascular  before  the 
atrophy,  the  grey  tint  is  generally  deepest.  The  vessels  are  but  little 
narrowed.  Occasionally  there  are  slight  signs  of  congestion  in  the 
early  stage,  and  then  some  tissue  of  gelatinous  aspect  may  develop  in 
the  disc,  and  the  vessels  may  appear  somewhat  compressed.* 

The  course  of  the  atrophy  is  usually  slowly  progressive,  and  in  most 
oases  it  ends  in  total  or  almost  total  blindness.  It  may,  however, 
cease  to  progress,  and  even  undergo  slight  improvement.  The  onset 
is  not  always  gradual ;  sometimes  sight  fails  considerably  in  the 
course  of  a  few  days,  without  ophthalmoscopic  changes  to  account  for 
it.  I  have  met  with  one  such  case  in  which  double  temporal  hemianopia 
indicated  disease  at  the  optic  chiasma.  In  such  cases  the  secondary 
interstitial  process  probably  assumes  inflammatory  intensity  (see 
p.  443).  The  atrophy  is  often  more  advanced  in  one  eye  than  in  the 
other,  and  very  rarely  one  eye  only  suffers.  Flashes  of  light  are 
occasionally  experienced  in  the  course  of  the  atrophy,  comparable  to 
the  lightning  pains  in  the  limbs,  but  they  are  not  common. 

Deafness,  having  the  characters  of  nerve-deafness,  is  met  with  in 
some  cases,  sudden  or  gradual  in  onset,  transient  or  lasting.  The 
persistent  deafness  has  been  attributed  to  an  atrophy  of  the  auditory 
nerve,  analogous  to  that  of  the  optic  nerve,  but  certainly  in  many 

*  Further  details  will  be  found  in  *  Medical  Ophthalmoscopy.' 


458  SPINAL    COED. 

cases  witliout  sufficient  reason.*  Only  when  tliere  is  a  progressive 
limitation  of  the  range  of  hearing,  analogous  to  the  peripheral 
limitation  of  the  field  of  vision,  are  we  justified  in  assuming  nerve 
atrophy.  I  have  seen  two  cases  of  this  kind.  In  each  there  was 
also  optic  nerve  atrophy,  and  the  other  symptoms  of  tabes  were 
distinct.f  Attacks  of  vertigo  are  frequent  in  the  cases  attended  by 
deafness,  and  seem  to  depend  on  the  disturbance  of  the  labyrinth  or 
auditory  nerve,  and  to  be  thus  connected  only  indirectly  with  the 
pr.mn.ry  disease.  But  chronic  slight  vertigo  from  this  cause  increases 
very  much  the  tabetic  unsteadiness.  Anosmia  occasionally  occurs,  and 
is  probably  not  infrequent.  It  is  apparently  due  to  an  atrophy  of  the 
olfactory  nerve  similar  to  that  of  the  optic. 

The  functions  of  the  other  cranial  nerves  are  not  often  affected. 
Pains  may  be  felt  in  the  region  of  the  fifth  nerve,  and  there  is  occa- 
sionally loss  of  sensation  in  some  part  of  the  area  supplied  by  it,  on 
one  side  or  both.  Unilateral  atrophy  of  the  tongue  has  been  sometimes 
noted.  In  the  larynx,  besides  the  spasm  to  be  presently  described, 
paralysis  of  the  vocal  cords  is  met  with,  both  as  an  isolated  symptom 
and  also  after  attacks  of  spasm  have  occurred  for  a  long  time.  Both 
posterior  crico-arytsenoids  may  be  paralysed,  and  in  one  case  paralysis 
of  one  posticus  occurred  very  early  in  the  disease  (Eemak). 

Sphincters. — The  functions  of  the  bladder  are  frequently  deranged; 
there  may  be  either  sluggish  micturition  or  a  tendency  to  incontinence, 
and  very  often  the  bladder  is  not  perfectly  emptied.  These  symptoms 
frequently  occur  early  in  the  disease,  and  it  may  be  indeed  only  for 
them  that  the  patient  seeks  medical  advice.  The  retention  may 
become  absolute,  or  there  may  be  overflow  incontinence ;  there  is 
rarely  paralytic  incontinence.  The  imperfect  contraction,  even  when 
slight,  may  have  serious  effects.  The  residual  urine  tends  to  decom- 
pose, and  gradually  induces  over- distension ;  cystitis  is  apt  to  occur, 
and  secondary  renal  disease  may  develop  insidiously,  and  is  a  not 
uncommon  cause  of  death  when  the  difficulty  in  micturition  has  been 
insignificant.  Slight  intermittent  febrile  disturbance  is  a  frequent 
consequence  of  this  bladder  incompetence  ;  it  may  cease  entirely  if  the 
catheter  is  regularly  used.  The  sphincter  ani  is  often  also  weak,  so 
that  a  loose  stool  can  be  retained  with  difficulty;  but  its  paralysis 
rarely  reaches  a  high  degree.     Constipation  is  extremely  common. 

*  It  is  very  difficult  to  distinguish  an  afEection  of  the  nerve  from  one  of  the 
labyrinth  independent  of  the  nerve.  Even  in  a  case  of  bilateral  deafness  coming 
on  in  the  course  of  tabes,  in  which  the  patient  could  hear  only  a  loud  voice,  and 
deep  notea  better  than  high  ones,  in  wliieh  atrophy  had  been  diagnosed,  Lucae  found 
calcareous  masses  in  eacli  of  the  labyrinths,  and  the  auditory  nerves  were  quite 
normal. 

+  In  one,  only  notes  between  E^  and  D  could  be  heard.  In  the  other  the  patient, 
when  first  examined,  was  absolutely  deaf  to  notes  above  E"  and  below  Gj.  A  few 
months  later  the  restriction  had  confined  the  range  of  hearing  to  the  octave  in  the 
treble  clef  between  E'^  and  E^,  even  EZ(i  being  inaudible.  Thus  the  loss  occurred: 
chiefly  fiom  below  upwards.     Ultimately  no  note  could  h'-  licjud. 


LOCOMOTOR    ATAXY.  459 

Vaso-motor  and  trophic  disturbances  constitute  an  important  group 
of  symptoms,  and  may  reach,  a  considerable  degree  without  a  coinci- 
dent increase  in  the  other  symptoms.  Local  sweating  has  been  noted, 
confined,  for  instance,  to  the  palms  and  soles,  or  to  one  side  of  the 
head.  The  ecchymoses  and  altered  growth  of  hair,  in  connection  with 
attacks  of  pain,  have  been  already  mentioned.  Pigment  may  disap- 
pear from  the  skin  and  hair  in  isolated  spots.  Herpes  of  the  skin  is 
not  uncommon,  and  usually  occurs  in  association  with  attacks  of  pain. 
A  pecuKar  association  was  present  in  one  case;  attacks  of  limited 
scrotal  pain  were  accompanied  by  a  "  rash  "  on  the  foreskin.  The 
epidermis  of  the  sole  becomes  thickened ;  blisters  readily  form  beneath 
it,  and  may  lead  to  indolent  ulcers.     The  curious  affection  known  as 


^-: 


Fig.  121. — Perforating  ulcer  of  the  foot  in  tabes.     (From  a  SalpStri&re 
photograph.) 

"  perforating  ulcer  of  the  foot "  has  been  found  to  be  almost  confined 
to  cases  of  tabes.  Troublesome  ulceration  of  the  toes,  especially 
about  the  nails,  is  also  occasionally  met  with,  and  may  necessitate 
amputation.  The  growth  of  the  nails  of  the  feet,  and  sometimes 
of  those  of  the  hands,  may  be  changed ;  they  may  be  thickened,  and 
the  surface  furrowed  or  irregular.  The  nails  may  even  fall  off  and  be 
slowly  renewed.  The  teeth  sometimes  decay  quickly,  and  may  drop 
out.  One  patient,  who  had  previously  lost  many  of  his  nails,  found 
that  all  the  teeth  of  the  upper  jaw  fell  out  in  the  course  of  three  days, 
without  any  pain  or  decay.  Wasting  of  the  muscles  occurs  only  as  a 
complication  in  the  later  stage  of  the  disease. 

Changes  in  the  nutrition  of  the  joints  and  bones  have  attracted 
much  notice  since  attention  was  directed  to  them  by  the  careful 
investigations  of  Charcot,  whose  name  is  sometimes  connected  with  the 
condition.  They  are  not  very  common,  but  are  sufficiently  frequent 
and  well  marked  to  place  their  relation  to  the  disease  beyond  doubt. 
The  bones  may  become  brittle,  and  may  break  readily,  in  so-called 
"  spontaneous  "  fractures.  The  process  of  union  is  attended  with  the 
formation  of  a  large  amount  of  callus.  Sometimes  there  is  also  ossi- 
fication or  calcification  of  the  structures  adjacent  to  injured  bones, 
and  even  of  the  fibrous  tissues  independent  of  the  joints,  sudx  as  tlid 


460  SPJXAL    CORD. 

intenmiscular  and  subcutaneous  tissues.  These  may  be  rapidly  in- 
flamed, and  extremely  quick  swelling  may  occxir,  followed  by  indura- 
tion. As  the  inflammation  subsides,  there  is  a  remarkable  tendency 
for  ossification  to  occur  even  in  the  newly  formed  inflammatory  tissue 
beneath  the  skin.  Such  subcutaneous  changes  occur  most  frequently 
in  the  foot  or  thigh,  especially  near  joints,  but  sometimes  in  the 
deeper  fibrous  tissues  between  the  muscles.  In  the  joints  the 
changes  consist  of  erosion  of  the  cartilage,  wasting  of  the  heads  of 
the  bones,  and  ossification  of  the  ligaments ;  sometimes  irregular  bony 
growths  form,  and  occasionally  the  head  or  shaft  of  a  long  bone 
may  undergo  an  extraordinary  increase  in  size  by  the  development  of 
new  osseous  tissue  on  the  surface.  The  joint  changes  may  thus 
assume  an  atrophic  or  hypertrophic  character,  and  the  movement  may 
be  either  too  free  or  too  limited.  Wasting  of  the  head  of  the  femur 
often  renders  dislocation  of  the  hip  easy.    With  such  changes,  and  often 


Fig.  122. — Licomotor  ataxy,  painless  swelling  of  elbow-joint  after  a 
fall  on  it.  One  condyle  of  the  humerus  and  olecranon  were  found, 
after  death,  to  have  been  broken  off.  Several  separate  pieces  of 
new  bone  had  formed  in  the  capsule  of  the  distended  joint. 

when  they  are  sUght  in  degree,  there  may  be  great  effusion  within  tlie 
joints  and  oedema  outside  them.  The  larger  joints  are  those  most 
commonly  diseased — ^knee,  hip,  ankle,  elbow, — but  the  small  joints  of 
the  fingers  have  been  known  to  suffer  (Westphal).  The  relative  fre- 
quency with  which  the  chief  joints  are  affected '  is  approximately 
indicated  by  the  following  percentage :  * — ^knee,  46 ;  hip,  20 ;  shoulder, 
11 ;  tarsus,  8 ;  elbow,  5 ;  ankle,  4.  They  often  occur  during  the 
pre-ataxic  stage.  The  lesions  are  sometimes  excited  by  injury,  and 
the  extraordinary  changes  that  may  follow  traumatic  influences,  or 
occur  alone,  afford  a  conclusive  indication  that  abnormal  trophic  in- 
fluences are  at  work.  Pig.  122  represents  the  arm  of  a  man  who  fell 
and  struck  his  elbow,  fracturing  the  olecranon  and  condyle.  This  was 
followed  by  extreme  painless  swelling,  and  bony  masses  could  be  felt, 
gradually  increasing  in  size.  After  death  the  fractures  of  the  bones 
were  found,  and  masses  of  new  bone  had  formed  in  the  capsule  of 
the  joints.     The  arthropathic  changes  occur  also,  however,  without 

*  Obtained  by  combining  the  collections  of  cases  of  Weizsacker  (Bruns'  *  Beitr, 
K.  kL  Chir.,'  1887)  and  Rotter  ('Arch.  f.  kl.  Chir.,'  Bd,  xxxvi^ 


LOCOMOTOR   ATAXY 


461 


injury,  and  are  probably  due  to  disease  of  the  nerves,  which  not  only 
has   a  direct    trophic  influence,   but    also    renders    the    structures 

insensitive  to  the  injurious  effects 
of  prolonged  tension  or  pressure 
(Rotter).  When  inco-ordination  has 
developed,  the  variations  in  the  mus- 
cular contractions  involve  varying 
strain  on  the  ligaments,  especially  in 
the  case  of  joints,  as  the  knee,  that 
depend  for  part  of  their  su23port  on 


— 

<; 

i 

P 

m 

%' 

C    b 

:< 

'^:' 

% 

% 

-^^. 

%'~ 

;;.|y:.x. 

Fia.  123. — Locomotor  ataxy;  retro- 
flexion of  knee-joints. 


"^ 


Fia.  124.— Tabetic  foot,     (From  a  photo- 
graph by  Mr.  Marriott,  B.Sc.) 


the  musctdar  tendons  that  pass  by  them.  The  knee-joint  may  thus 
become  so  lax  that  when  the  patient  stands,  the  joint  may  become 
retroflexed,  as  in  Fig.  123.  No  doubt  also  this  is  the  result  to  some 
extent  of  the  want  of  tone  in  the  muscles,  which  has  been  called  by 
Frenkel  hypotonie  musculaire.  Changes  in  the  tarsal  bones  and  articu- 
lations may  cause  the  foot  to  become  flat,  with  a  projection  inwards  or 
backwards  of  the  tarso-metatarsal  articulations,  and  of  the  (often 
enlarged)  tarsal  bones.  The  condition  has  been  called  the  "tabetic 
club-foot "  by  Charcot  and  Fere.* 

In  some  cases  there  is  painless  loss  of  teeth,  and  this  may  be  accom- 
panied by  the  presence  of  troublesome  ulceration  of  the  mouth,  re- 
garded by  some  as  analogous  to  the  perforating  tdcer  of  the  foot. 

Visceral  symptoms  of  peculiar  character  occur  in  many  cases  of 
tabes. t  They  consist  almost  entirely  of  paroxysmal  disturbance  of 
function,  usually  attended  with  great  paia,  and  have  been  termed, 

*  The  trophic  changes  in  bone  and  joint  have  been  regarded  by  some  as  simple 
chronic  osteo-arthritis,  without  causal  relation  to  the  nerve  disease  in  their  subjects, 
but  the  evidence  is  altogether  opposed  to  this  view.  The  arguments  that  can  be 
adduced  in  support  of  it  will  be  found  in  some  of  the  speeches  delivered  at  a  dis- 
cussion at  the  Clinical  Society,  Nov.  and  Dec,  1885. 

t  The  occurrence  of  attacks  of  vomiting,  sometimes  early  in  the  disease,  wag 
pointed  out  by  Topinard,  but  thought  by  him  to  be  a  mere  complication.  (Topinard, 
•  De  I'ataxie  locomotrice,'  Paris,  1864,  p.  273.) 


462  SPINAL  CORD. 

by  the  Frencli,  crises,  witli  qualifying  adjectives  according  to  the 
seat  of  tlie  symptom.  There  is  a  tendency  to  over-elaborate  tliia 
terminology.  The  most  frequent  seat  of  such  disturbance  is  the 
stomach,  and  these  attacks  are  called  gastric  crises.  They  consist  of 
paroxysms  of  severe  gastric  pain,  felt  in  the  epigastrium  and  often 
passing  through  to  the  back.  The  pain  is  accompanied  by  vomiting, 
with  or  without  nausea.  The  vomiting  is  often  incessant,  and  is  first 
of  food,  then  of  clear  liquid,  which  may  be  very  abundant ;  ultimately 
bile  is  vomited,  and  sometimes  blood,  which  may  be  altered  in  colour ; 
it  has  been  ascribed  to  simultaneous  vaso-motor  disturbance  in  the 
mucous  membrane.  Retardation  or  irregularity  of  the  action  of  the 
heart  has  sometimes  attended  such  an  attack,  and  rarely  pyrexia  has 
been  noted.  I  have  known  frequent  hiccough  to  accompany  the 
vomiting.  Such  an  attack  lasts  for  some  hours  or  days,  and  then 
subsides,  to  recur  in  a  few  weeks.  During  the  intervals  the  functions 
of  the  stomach  may  be  performed  in  a  perfectly  normal  manner. 
Although  pain  and  vomiting  usually  occur  together,  some  patients 
'have  attacks  of  pain  without  vomiting,  and  in  others  there  is  vomiting 
without  pain.  It  may  occur  on  first  rising  in  the  morning,  and  be 
relieved  by  recumbency.  Rarely  there  is  nausea  alone.  A  temperate 
man,  in  the  early  stage  of  tabes,  suffered  during  three  years  from 
frequent  attacks  in  which,  for  several  days,  he  had  intense  nausea  each 
morning,  passing  away  in  the  afternoon,  and  succeeded  in  the  evening 
by  an  inordinate  craving  for  food.  He  never  vomited  unless  he  made 
himself  do  so,  in  the  vain  hope  of  thus  obtaining  relief.  After 
two  years,  attacks  of  laryngeal  spasm  were  added  to  the  nausea. 
Occasionally  gastric  attacks  coincide  with  paroxysms  of  pain  in  the 
trunk. 

I'he  chief  intestinal  disturbance  is  constipation,  but  paroxysmal 
diarrhoea  has  been  supposed  to  be  of  vaso-motor  origin,  and  connected 
•with  the  disease  (Pierret).  Paroxysms  of  recta]  pain  (rectal  crises) 
may  occur,  and  may  be  accompanied  by  distressing  tenesmus,  some- 
times by  a  sensation  as  if  there  were  a  foreign  body  in  the  rectum. 
In  rare  cases  paroxysms  of  pain  have  precisely  the  character  of 
attacks  of  renal  colic  {nephralgic  crises),  or  are  felt  in  the  neck  of  the 
bladder  or  along  the  urethra  or  at  the  meatus  {vesical  or  urethral 
crises).  These  may  be  attended  by  an  intense  desire  to  micturate, 
although  the  bladder  may  be  empty  and  only  a  few  drops  of  urine 
may  be  expelled.  In  one  patient  such  pain  was  repeated  several 
times  an  hour,  and  each  attack  lasted  for  several  days.  Blood  may  be 
passed  after  an  attack. 

Laryngeal  crises  are,  perhaps,  after  those  of  the  stomach,  the  most 
common.  They  vary  much  in  character.  The  most  common  form  is 
a  true  laryngeal  spasm,  with  noisy  inspiration  and  expiration,  cough, 
and  often  considerable  dyspnoea.  The  paroxysms  may  resemble  those 
of  whooping-cough  or  of  laryngismus  stridulus,  and  seem  to  be  due  to 
spusin  of  the  adductors.     Pressure  on  the  superior  laryngeal  nerve  at 


LOCOMOTOR   ATAXY.  463 

its  entrance  into  tlie  larynx,  or  on  the  trachea,  or  the  introduction  of 
a  sound,  ■will  sometimes  induce  an  attack.  The  spasm  may  last  for  a 
quarter  of  an  hour  or  for  some  hours,  but  rarely  continues  so  long  as 
the  gastric  crises.  In  most  if  not  all  these  cases  of  adductor  spasm 
there  is  permanent  weakness  of  the  abductors,  which  when  consider- 
able constitutes  a  very  grave  complication.  Whenever  there  is  a 
history  of  laryngeal  spasm,  the  respiratory  sound  should  be  carefully 
observed  to  detect  any  inspiratory  stridor.  In  one  recorded  case  the 
spasm  spread  to  the  pharynx,  making  swallowing  impossible ;  a  -violent 
attack  extended  to  the  muscles  of  respiration,  and  the  patient  died 
asphyxiated.  Death  from  these  attacks  is,  however,  rare.  Paroxysms  of 
rough  cough  have  been  termed  ''bronchial  crises;"  in  one  case  such 
attacks  ceased  when  the  patient  began  to  suffer  from  gastric  crises. 
Such  paroxysmal  cough,  sometimes  ending  with  a  "  whoop,"  is  more 
frequent  than  definite  laryngeal  spasm.  The  definite  crises  are,  how- 
ever, often  early  symptoms,  and  they  may  continue  for  many  years.  I 
have  seen  a  patient,  still  in  the  first  stage,  who  gave  a  history  of 
gastric  crises  during  the  preceding  eighteen  years.  Anginous  attacks 
have  also  been  described.  Acceleration  of  the  pulse  is  common,  and 
cardiac  disease,  especially  aortic,  by  no  means  rare. 

Comparable  to  these  paroxysmal  visceral  disturbances,  although 
very  different  in  character  and  more  alarming  in  aspect,  are  cerebral 
symptoms ;  but  these  are,  fortunately,  very  rare.  They  resemble  the 
attacks  that  are  common  in  general  paralysis  of  the  insane,  and  con- 
sist of  transient  apoplectiform  seizures,  of  transient  hemiplegia,  or  of 
convulsions,  general  or  one-sided.  They  may  occur  early  in  the  course 
of  the  disease.  The  alarm  they  occasion  is  not  without  foundation, 
since  death  has  been  known  to  occur  during  an  attack  of  apoplectic 
aspect.  Occasionally  an  actual  lesion  occurs  (probably  thrombotic 
softening),  causing  persistent  hemiplegia,  which  is  probably  only 
coincident  with  the  chronic  disease  of  the  nervous  system.  Paroxysms 
of  vertigo  sometimes  occur,  but  their  nature  is  uncertain.  They  pro- 
bably frequently  depend  on  derangement  of  the  auditory  nerve  or 
labyrinth.  Cerebral  symptoms  may  also  be  superadded,  especially  ia 
cases  which  terminate  as  general  paralysis. 

Course  and  Termination. — It  is  convenient  to  divide  the  course  of 
the  disease  into  three  stages : — (1)  In  which  there  is  no  alteration  in 
gait,  the  common  indications  of  the  malady  being  the  loss  of  the 
knee-jerk  and  the  pains,  often  associated  with  loss  of  the  light-reflex 
of  the  iris,  and  unsteadiness  on  standing  with  the  feet  together  and 
eyes  shut.  (2)  That  in  which  there  is  distinct  affection  of  gait,  slight 
or  considerable,  but  in  which  the  patient  is  stiU  able  to  walk,  alone  or 
with  the  aid  of  one  or  two  sticks.  .  (3)  In  which  walking  is  possible 
only  with  the  aid  of  another  person,  or  it  is  impossible  for  the  patient 
to  walk  or  to  stand.  The  first  stage  is  not  always  present ;  inco-ordi- 
nation  may  be  developed  simultaneously  with  the  loss  of  the  knee- 
jerk,  as  one  of  the  earliest  symptoms. 


4G4  SPINAL   CORD. 

The  course  of  the  symptoms  is  extremely  Yariable.  TBe  inco-ordi- 
nation  usually  supervenes  gradually  in  tlie  course  of  months,  but  it 
sometimes  develops  very  rapidly.  I  have  kaown  it  to  become  extreme 
in  the  course  of  a  fortnight  from  its  onset,  after  the  first  stage  had 
existed  for  a  considerable  time.  The  epithet  "progressive,''  given  to 
the  malady  by  Duchenne,  expresses  a  characteristic  of  a  large  propor- 
tion of  the  cases  in  which  ataxy  is  developed,  and  he  kaew  no  other 
cases  of  the  disease.  The  inco-ordination,  once  developed,  often 
increases  slowly  or  rapidly,  until  it  reaches  a  moderate  or  a  consider- 
able degree,  and  the  sensory  loss  shows  a  like  tendency.  The  local 
onset  of  the  ataxy  seems  to  be  sometimes  excited  by  a  peripheral 
influence,  such  as  injury  ;  in  one  case  an  injury  to  the  left  hand  was 
followed  by  inco-ordination  in  that  limb,  afterwards  general  and  cha- 
racteristic. Still  more  common  is  a  rapid  increase  in  the  symptoms  after 
such  a  disease  as  influenza,  or  in  consequence  of  alcoholic  excess.  In 
such  cases  careful  inquiry  will  generally  elicit  evidence  of  preceding 
symptoms.  The  pains  seldom  correspond  to  the  other  symptoms  in 
degree  or  course.  They  are  often  very  severe  in  the  early  period  of 
the  disease,  and  may  either  lessen  or  continue  unchanged  as  the  loss 
of  sensation  develops.  Their  persistence,  even  in  severe  degree,  does 
not  show  progress  in  the  disease  ;  once  set  up,  they  may  continue  for 
years  without  any  increase  in  the  other  symptoms,  and  seem  in  this  to 
be  analogous  to  neuralgia,  being  evidence  only  of  functional  activity 
in  the  altered  nerves. 

The  power  of  recognising  the  first  stage  of  the  disease,  which  we 
owe  to  Westphal's  discovery  of  the  loss  of  the  knee-jerk,  has  enlarged, 
and  in  enlarging  has  to  some  extent  modified,  our  conception  of  the 
general  tendency  of  the  disease.  It  is  exceedingly  common  for  the 
first  stage  to  remain  stationary  for  a  long  time,  for  ten,  twenty,  and 
eveu,  in  one  case  I  have  known,  for  twenty-five  years,  if  the  duration 
of  the  lightning  pains  may  be  accepted  as  proof  of  its  existence.  Wl ;  en 
all  cases  are  taken  together,  and  if  the  patients  are  subjected  to  careful 
treatment,  the  disease  probably  shows  a  progressive  tendency  in 
less  than  one  half  of  the  cases. 

The  other  symptoms  also  do  not  always  progress  pari  passu.  Sen- 
sibility may  fail  although  inco-ordination  does  not  increase.  Con- 
versely, the  ataxy  may  become  greater,  although  sensation  remains  the 
same,  and  even  when  it  shows  a  distinct  improvement.  To  this  we 
shall  return  in  considering  the  pathology  of  the  disease.  The  contrast 
between  the  course  of  different  symptoms  in  some  cases  is  very 
marked.  If  optic  nerve  atrophy  develops,  the  spinal  symptoms  often 
remain  stationary,  and  this  is  also  true  of  visceral  crises;  I  have 
known  gastric  crises  to  cease  when  inco-ordination  came  on.  In  one 
patient  aU  the  symptoms  of  tabes  passed  away,  optic  nerve  atrophy 
ceased  to  advance,  and  even  the  knee-jerk  returned,  when  the  patient 
became  insane.  The  atrophy  of  the  optic  nerves,  although  generally 
progressive  to  blindness,  also  may  become  arrested.    It  may  remain 


LOCOMOTOR   ATAXY.  465 

limited  to  o^''  Cy c-,  and  even  when  bilateial  and  very  definite,  it  may 
cease  to  increase. 

The  rate  of  increase  in  the  symptoms  of  tabes,  when  this  occurs, 
varies  much.  Sometimes  it  is  so  slow  that  the  ataxy  is  considerable 
only  at  the  end  of  several  years.  On  the  other  hand,  it  may  be  so 
rapid  that  in  a  few  months  the  patient  is  scarcely  able  to  walk.  More 
commonly  the  course  is  variable ;  periods  in  which  the  disease  is  almost 
stationary  alternate  with  others  in  which  the  symptoms  increase 
rapidly.  The  exacerbations  may  seem  spontaneous,  or  may  be  dis- 
tinctly excited  by  some  prejudicial  influence— a  chill,  a  fall,  or  some 
excess,  alcholic  or  sexual.  They  are  sometimes  very  acute ;  a  marked 
change  may  occur  in  the  course  of  a  few  days,  or  even  in  a  few  hours. 
Thus  I  have  known  a  patient  to  pass,  in  the  course  of  twenty-four 
hours,  from  a  condition  in  which  he  could  walk  fairly  well  to  one  in 
which  he  could  scarcely  stand.  The  symptoms  suggested  that  local 
myelitis  had  occurred  in  the  degenerating  cord.  Muscular  power  may 
lessen  in  such  a  sudden  exacerbation,  or  the  increase  may  be  confined 
to  the  special  symptom  of  the  disease — the  ataxy.  The  conditions 
thus  developed  may  pass  away  again,  leaving  the  patient  on  a  little 
lower  level  than  before,  but  they  often  persist  without  any  considerable 
recovery. 

There  is  nothing  in  the  nature  of  the  disease,  in  most  cases,  to 
produce  death.  The  only  direct  effect  of  the  malady,  which  has  ended 
life,  is  laryngeal  spasm  or  paralysis.  Even  the  gastric  symptoms, 
prostrating  as  they  are,  are  never  fatal.  Patients  who  have  reached 
the  third  stage  have  lived,  bedridden,  for  twenty  years.  Death  often 
results  from  intercurrent  maladies,  some  of  which  are  more  dangerous 
to  these  sufferers  than  to  others  because  they  may  develop  painlessly, 
and  attain  a  serious  degree  before  their  existence  is  suspected.  But 
many  patients  die  from  indirect  results  of  the  disease.  Kidney  com- 
plications are  the  most  common ;  they  often  develop  insidiously,  and 
only  manifest  themselves  when  life  is  in  peril,  as,  for  instance,  by 
acute  febrile  symptoms,  the  exact  nature  of  which  may  be  unsuspected 
until  the  urine  is  examined.  I  have  known  a  patient,  still  in  the  first 
jStage,  die  thus  after  a  few  days'  illness.  Whenever  obscure  pyrexia  is 
met  with  in  diseases  of  the  spinal  cord,  interference  with  the  function 
of  the  kidneys,  due  to  retention  of  residual  urine,  should  always  be 
suspected  and  looked  for.  Bedsores  and  pyaemia  occasionally  cause 
death,  although  far  less  frequently  than  in  most  other  diseases  of  the 
spinal  cord. 

Complications. — Occurring,  as  most  cases  do,  in  syphilitic  subjects, 
tabes  is  sometimes  complicated  by  true  syphilitic  lesions  of  the  brain 
or  ccrd,  generally  the  later  lesions,  such  as  gummata,  or  sudden  palsy 
from  the  disease  of  a  cerebral  vessel. 

Acute  or  subacute  myelitis  may  occur  during  the  course  of  the 
disease  with  characteristic  symptoms.     Such  a  complication  must  not 

VOL.  I.  30 


466  SPINAL   CORD. 

be   confounded  witli  the   simple  acute  exacerbation  in  tlie  special 

symptoms,  which,  sometimes  occurs  with  remarkable  rapidity. 

Other  system  diseases  of  the  cord  may  develop  as  complications  of 
tabes.  The  lateral  columns  may  be  diseased  with  the  dorsal  posterior 
columns,  giving  rise  to  "  ataxic  paraplegia,"  but  this  is  a  distinct 
affection,  and  will  be  so  described.  Another  occasional  complication  is 
muscular  atrophy,  both  the  local  atrophy  already  mentioned  (espe- 
cially frequent  in  the  tongue),  and  general  atrophy,  such  as  is  met 
with  in  the  pure  spinal  form.  Thus  a  gentleman,  who  had  had 
hemiplegia  from  syphilitic  vascular  disease,  developed  the  first  stage 
of  tabes — pains,  loss  of  Imee-jerk,  loss  of  the  iris-reflex,  and  slight 
unsteadiness.  He  had  two  courses  of  mercurial  treatment  at  Aix-la- 
Chapelle,  and  at  the  end  of  the  second,  atrophy  began  in  the  muscles 
of  the  hands,  and  spread  during  two  years  until  almost  all  the 
muscles  of  the  arms,  shoulders,  and  back  became  extremely  wasted. 
Local  muscular  wasting  (according  to  the  researches  of  Dejerine*) 
is  usually  the  result  of  degeneration  of  the  peripheral  nerves ;  it 
is  generally  symmetrical.  The  extensive  wasting,  as  in  the  case 
just  mentioned,  is  probably  due,  as  a  rule,  to  degeneration  in  the 
anterior  cornua  of  the  spinal  cord. 

In  rare  cases  the  legs  present  the  symptoms  of  tabes,  while  in  the 
arms  there  is  the  jerky  inco-ordination  of  disseminated  sclerosis,  and 
the  co-existence  of  the  two  lesions  has  been  demonstrated,  but  is  very 
rare. 

Another  very  important  and  frequent  complication  of  tabes  is 
general  paralysis  of  the  insane.  The  two  diseases  have  many  alHances. 
It  is  probable  that  syphilis  is  the  chief  cause  of  general  paralysis,  as 
well  as  of  tabes.  E-eflex  iridoplegia  is  common  in  both  diseases.  The 
two  maladies  are  often  combined,  and  the  symptoms  of  one  or  the 
other  may  preponderate.  Thus  many  general  paralytics  present  sym- 
ptoms of  tabes,  and  its  characteristic  lesion  is  found  after  death.  On 
the  other  hand,  cases  of  tabes  may  present  slight  symptoms  of  general 
paralysis,  perhaps  only  slight  optimism  and  mental  weakness,  which 
may  remain  subordinate,  or  may  increase  to  a  pronounced  and  pre- 
ponderant degree.  It  may  be  difficult  to  say  in  which  category  a  case 
should  be  placed.  It  is  sometimes  said  that  the  disease  may  com- 
mence as  ataxy,  and  may  change  to  general  paralysis,  but  a  more 
correct  expression  of  the  facts  is  the  co-existence  of  the  two  affections, 
and  the  dominance  of  the  symptoms  of  one  or  the  other. 

Of  complications  of  tabes  outside  the  nervous  system,  besides  the 
indirect  consequences  of  the  malady,  valvular  heart  disease  is,  perhaps, 
the  most  important,  and  usually  develops  without  any  of  the  usual 
antecedents,  and  at  an  age  at  which  degeneration  is  unlikely.  It 
is  not  improbable  that  the  coincidence  is  really  one  of  cause,  the 
Talvular   disease  being  the  result  of    a   syphilitic  process.     Other 

*  Dejerine,  '  Revue  de  Med.,'  1889. 


LOCOMOTOR   ATAXY. 


467 


complications  are  so  rare  as  only  to 
desei-ve  notice  when  a  causal  con- 
nection can  be  traced  or  reasonably 
suspected.  Coiacident  glycosuria  has 
been  ascribed  to  an  affection  of  the 
medulla  oblongata  (or  may  be  pro- 
duced through  the  cervical  spinal 
cord),  but  it  is  extremely  rare.  But 
sclerosis  of  the  posterior  columns 
has  been  shown  by  Williamson  to 
occur  in  diabetes.* 

PATHOLOGiOAii  Anatomt. — In  most 
cases  the  spinal  cord  presents  changes 
visible  to  the  nakod  eye.  The  poste- 
rior columns  have  a  grey  translucent 
appearance,  which  is  due  to  the  loss  of 
the  nerve-fibres,  and  to  an  increase  of 
the  connective  tissue  ("  grey  degene- 
ration ").  The  whole  of  the  posterio- 
columns  may  be  thus  changed,  or  only 
parts  of  them,  the  distribution  of  the 
visible  alteration  being  that  of  the 
histological  changes  revealed  by  the 
microscope  and  presently  to  be  de- 
scribed. In  the  hardened  cord  the 
difference  of  tint  in  the  diseased  areas 
is  even  more  distinct  than  it  is  in  the 
fresh  organ.  The  posterior  columns 
are  also  smaller  than  normal,  because 
the  bulk  of  the  connective  tissue  is 
less  than  that  of  the  normal  fibres: 
hence  the  shape  of  the  cord  is  slightly 
changed. 


Fia.  125. — -Locomotor  ataxy  involving:  the 
legs  only.  Sclerosis  of  the  whole  posterior 
columns  in  the  lumhar  region,  gradually 
hecoming  limited,  in  the  lower  dorsal  re- 
gion, to  the  root-zone  and  posterior  median 
column.  The  latter  only  is  affected  in  the 
upper  half  of  the  cord(aseendino-  degenera- 
tion). There  isalso  coni^picuous  degeneration 
in  the  antero-lateral  ascending  tract.  This 
is  rather  greater  on  tlie  left  side,  while  the 
post.-median  degeneration  is  greater  on  the 
right,  in  harmony  with  the  fact  that  the 
latter  consists  of  fihres  which  do  not  decus- 
sate. 


•  *  Lancet.'  1894. 


468 


SPINAL    COED. 


In  a  section  of  the  cord  stained  with  carmine  or  similar  reagent, 
and  examined  under  the  microscope,  the  affected  areas  are  conspicuous 
by  the  deep '  staining  of  the  connective  tissue.  The  position  of  the 
excess  of  this  tissue,  of  the  "  sclerosis,"  as  it  is  term.ed,  indicates  the 
place  in  which  the  nerve-fibres  have  degenerated.  In  the  most 
frequent  condition,  in  which  the  legs  only  are  affected,  the  sclerosis 
occupies  the  whole  of  the  columns  in  the  lumbar  region  (Fig.  125),  but 
it  is  often  slight  in  the  anterior  parts  of  the  postero- external  columns, 
which  may  even  be  free  from  sclerosis ;  also  in  the  middle  of  these 
columns  many  more  nerve-fibres  may  be  seen  than  elsewhere.  The 
sclerosis  is  most  dense  in  the  part  adjacent  to  the  posterior  cornu, 
through  which  the  posterior  root- fibres  run,  and  near  the  surface  of  the 
cord  (Fig.  125,  d  12).  Above  the  lumbar  enlargement  the  affection  of 
the  postero-external  columns  gradually  ceases,  but  the  degeneration  is 
intense  in  the  postero-median  columns,  and  has  the  distribution  of  an 
ascending  degeneration,  as  it  in  fact  is,  receding  from  the  commissure 
in  the  upper  cervical  region.  (Compare  Fig.  125  with  Fig.  77,  p.  214.) 
In  other  cases  the  external  band  of  sclerosis,  adjacent  to  the  cornu, 
continues  separate  through  the  dorsal  cord  and  even  through  the 
cervical  enlargement  (Fig.  126) ;  the  median  degeneration  then  extends 


Fio.  12fi, — Tabes  with  ataxy  of  the  arms  as  well  a<!  the  Ipgs.  Section 
of  cord  in  cervical  region,  showing  sclerosis  of  the  post.-med.  column 
ar  d  ront-zone  of  |iost.-ext.  col.     Degeneration  of  left  ant.  cornu. 


■ap  to  the  commissure  throughout  the  cervical  region,  just  as  does  the 
secondaTT'  degeneration  that  results  from  a  cervical  lesion  (see  Fig.  78, 
p.  216).  But  in  the  cervical  enlargement,  the  unaffected  area  in  the 
anterior  part  of  the  postero-external  column  is  usually  much  larger 
than  in  the  lumbar  enlargement. 

In  rare  cases,  of  severe  and  long  duration,  the  posterior  columns  ai-e 
occupied  bj'  connective  tissue  in  their  whole  extent  from  one  end  of  the 
cord  to  the  other.  A  few  fibres  of  the  posterior  roots  and  a  few 
vertical  fibres  near  the  commissure  may  alone  be  recognisable.  An 
instance  of  such  almost  complete  sclerosis  is  shown  in  Fig.  127. 

On  the  other  hand,  in  slight  cases,  in  which  the  disease  is  still  in  the 
first,  or  in  the  commencement  of  the  second  stage,  the  sclerosis  ia 
moderate  in  degree,  even  in  the  regions  of  the  cord  most  affected. 


LOCOMOTOR  ATAXY. 


469 


The  separation  of  the  sclerosed  areas  in  the  postero-extemal  and  the 

postero-median  columns  may  be 
distinct  throughout  the  cord, 
except  in  the  lumbar  enlarge- 
ment, where  the  median  degene- 
ration expands  into  the  external 
column.  The  sclerosis  of  the 
postero-external  column  is  then 
usually  limited  to  the  root- zone, 
and  varies  m  .width,  according 
to  that  of  the  area  through 
which  the  root-fibres  pass.  In 
the  dorsal  region  there  is  usu- 
ally a  narrow  band  of  sclerosis 
close  to  the  posterior  cornu ;  in 
the  lumbar  region  it  reaches 
almost  to  the  median  septum. 
In  some  instances,  however,  a 
very  slight  degree  of  sclerosis 
extends  from  the  most  affected 
tracts  through  the  rest  of  the 
columns,  and  is  generally  great- 
est in  their  posterior  half.  This 
condition  existed  in  the  very  in- 
structive case  shown  in  Fig.  128. 
The  intensification  of  this  dif- 
fuse sclerosis  in  the  root-zone 
and  median  columns  is  very  dis- 
tinct, although  the  affection  of 
the  latter  is  unusually  slight. 
There  is,  moreover,  in  the  dorsal 
region,  sclerosis  of  another  part 
of  the  postero-external  column 
— the  comma-shaped  tract  of 
short  fibres,  which  degenerates 
downwards  for  a  few  .inches 
below  a  transverse  lesion  of  the 
cord  (see  p.  219  and  Figs.  78 
and  110,  F).  It  is  most  distinct 
in  128  at  d  8,  but  can  be  traced 
up  to  the  cervical  region,  where 
its  fonn  is  changed. 

The  tract  of  fine  fibres  of  the 
posterior  root,  discovered  by 
Lissauer,  is  generally  diseased.* 

*  It  was  first  described  by  Lissauer 
in  connection  with  its  disease  in  tabes; 


Pia.  127.  —  Locomotor  ataxy:  extreme 
incO'Ordination  and  ansesthesia  in  both 
arms  and  legs;  the  posterior  columng 
are  sclerosed  throughout  the  cord  in 
their  entire  extent.  A,  upper  cervical, 
B,  C,  D,  dorsal,  E,  lumbar  retrions.  In 
D  there  is  also  some  degeneration  of  the 
intermediate  grey  substance. 


470 


SPINAL   CORD. 


The  antero-lateral  columns  may 
be  perfectly  normal,  even  wlien  tlie 
disease  of  the  posterior  columns 
is  great.  Often,  however,  in  old 
cases,  there  is  a  slight  general 
increase  of  the  connective  tissue 
throughout  the  cord.  Sometimes 
there  is  distinct  sclerosis  of  other 
definite  tracts,  e.  g.  of  the  ascend- 
ing antero-lateral  tract  (as  in  Fig- 
125),  or  of  the  direct  cerebellar 
tract.  In  rare  cases  there  is  a 
distinct  degeneration  of  the  pyra- 
midal tracts.  The  whole  periphery 
of  the  antero-lateral  column  is 
thus  occasionally  the  seat  of  scle- 
rosis, the  connective  tissue  ex- 
tending in  from,  the  pia  mater, 
which  is  usually  also  thickened; 
it  is  probable  that  this  condition 
may  be   solely  the  result  of  the 

Pr&.  128. — Logo,  ataxy;  sciiEROsis  op 
POSTBEiOE  COLTTMNS. — In  L  1  the  pos- 
terior portion  of  both  post.-ined.  col. 
and  post.-ext.  col.  is  densely  sclerosed, 
but  in  front  only  the  post.-med.  col.  At 
D  10  the  sclerosis  is  also  general,  but  is 
dense  only  in  the  root- zone  of  the  post.- 
ext.  col.  At  D  8  and  D  4  the  change 
is  sliglit,  except  in  three  areas,  the  root- 
zone,  the  middle  part  of  the  post.-med. 
col.,  and  the  comma-shaped  tract  in  the 
front  of  the  post.-ext.  col.  At  D  1  the 
comma-shaped  tract  is  narrow,  and  in 
C  7  it  merges  in  a  band  of  sclerosis, 
which  bounds  the  anterior  part  of  the 
post.-med.  col.  There  is  a  little  diffuse 
sclerosis  through  the  rest  of  the  column, 
and  in  the  first  dorsal  there  is  some 
degeneration  of  the  anterior  cornua. — 
Symptoins  of  the  first  stage  had  existed 
for  four  years  (lightning  pains,  slight 
analgesia,  loss  of  knee-jerk  and  iris-re- 
flex, gastric  crises,  optic  nerve  atrophy). 
A  few  weeks  before  death  the  legs 
gradually  became  paralysed,  and  the 
arms  ataxic ;  retention  of  urine  caused 
acute  kidney  mischief,  which  was  the 
immediate  cause  of  death.  No  Ipsion 
was  found  after  death  to  explain  the 
subacute  paralysis,  but  the  peripheral 
nerves  were  not  examined,  their  dis- 
ease being  then  unknown. 

see  '  Neur.  Cent.,'  1885,  p.  245 ;  also  ib., 
1886,  p.  419. 


LOCOMOTOR   ATAXY.  471 

primary  degeneration  of  the  nerve-fibres  even  "when  it  closely  simulates 
the  aspect  of  meniugitic  invasion.  The  cornu- commissural  and  septo- 
marginal tracts,  the  former  close  to  the  posterior  commissure  at  the 
most  anterior  part  of  the  posterior  columns,  the  latter  on  each  side  of  the 
posterior  median  septum,  are,  as  a  rule,  spared  in  tabes.  They  probably 
consist  of  endogenous  fibres,  connecting  different  levels  of  the  cord.* 

In  slight  cases  it  is  difiicult  to  distinguish  any  changes  in  the  grey 
matter  of  the  cord,  although  it  is  probable  that  there  is  some  atrophy 
of  the  nerve-cells  and  fibres  in  the  posterior  horn  in  many  cases. 
Various  structural  changes  can  be  discerned  in  more  advanced  cases 
(as  Lockhart  Clarke  first  showed),  and  the  posterior  commissure  may 
be  reduced  in  size.  The  posterior  vesicular  column  also  shows  distinct 
degeneration  in  most  cases.  Some  atrophy  of  the  plexus  of  fine  fibres 
contained  in  this  column  is  said  to  be  almost  invariable  (Lissauer), 
but  frequently  the  cells  and  vertical  fibres  waste,  and  sometimes  only 
a  few  shrunken  cells,  scattered  through  a  translucent  nucleated  tissue, 
may  remain.  The  atrophy  of  the  cells  is  said  to  be  especially 
associated  with  sclerosis  of  the  direct  cerebellar  tract;  also  (but  on 
slighter  evidence)  with  that  of  large  fibres  in  the  pyramidal  tract, 
which  do  not  degenerate  downwards.  When  the  cells  are  unaffected, 
although  the  intervening  substance  is  diseased,  the  cerebellar  tract 
may  be  normal,  and  the  fibres  passing  to  it  from  the  front  of  the 
vesicular  tract  may  be  unaffected.  The  degeneration  of  the  grey 
matter  often  extends  into  the  intermediate  region  between  the  cornua 
(Pig.  127,  b)  ;  the  intermedio-lateral  tract  of  cells  may  be  atrophied, 
and  occasionally  even  the  anterior  cornu  and  its  cells  suffer  in  a 
similar  way  (Figs.  126,  128,  D  1).  As  we  have  seen,  this  change  may 
be  the  cause  of  muscular  atrophy,  although  in  other  instances  the 
mascular  wasting  depends  on  alterations  in  the  motor  nerves. 

The  sclerosis  varies  considerably  in  its  histological  characters.  In 
slight  cases  there  is  merely  a  moderate  thickening  of  the  trabeculse 
and  their  branches.  The  larger  tracts  are  more  fibrous  than  normal, 
and  more  nuclei  are  seen  than  in  health.  Everywhere  the  new  growth 
of  connective  tissue  proceeds  chiefly  from  the  pre-existing  tracts, 
vessel- walls,  &c.  When  the  change  is  considerable  the  trabeculse  are 
very  broad,  and  areas  seem  to  consist  wholly  of  connective  tissue, 
fibrillated  and  nticleated.  In  old  cases  there  is  sometimes  little 
appearance  of  fibrillation  in  the  most  affected  region;  cells  and 
thickened  vessels  lie  in  a  granular  or  homogeneous  tissue.  From  the 
parts  most  changed  the  nerve-fibres  have  wholly  disappeared ;  in  other 
parts,  however,  close  examination  reveals  many  fibres  narrower  than 
normal,  and  many  stained  points  that  may  possibly  be  axis-cylinders. 
The  walls  of  the  vessels  are  usually  increased  in  thickness,  sometimes 
to  a  very  considerable  degree.  The  coat  next  the  endothelium  may  be 
thickened  and  contain  many  nuclei,  but  the  chief  increase,  in  most 
cases,  is  in  the  outer  coat  and  adventitia,  whence  the  tracts  of  tissue 
•  See  A.  Bruce,  « Brain,'  1897. 


472 


SPINAL    COED. 


pass  ofE  into  tlie  adjacent  region.  The  pia  mater  is  sometimes 
thickened  over  the  posterior  columns ;  tlie  vessels  in  it  may  then  be 
similarly  changed.  The  alteration  in  the  membrane  may  extend  over 
tbe  neighbouring  part  of  the  lateral  column,  and  even  around  tbe 
cord  (Fig.  129),  and,  as  already  stated,  septa  passing  from  it  into  the 
cord  may  be  thickened  in  the  circumferential  zone.  This  peripheral 
sclerosis  is  probably  the  result  of  three  distinct  processes,  the  relative 
share  taken  by  each  varying  in  different  cases :  (a)  a  primary  degene- 
ration of  the  peripheral  ascending  tracts;    (b)  a  process  of  chronic 


V 


PlO.  129. — Tabes,  Chronic  meningitis.  Cervical  region.  Sclerosis  of 
post-median  columns  and  slight  diffuse  sclerosis  of  the  cord,  least  in 
ant.  cols.,  dense  in  the  right  lateral  column.  Thickened  pia  mater, 
and  damage  to  subjacent  superficial  layer  of  the  cord. 

interstitial  myelitis,  spreading  from  the  pia  mater,  which  is  the  seat 
of  an  inflammatory  tissue  formation ;  this  may  cause  (c)  a  secondary 
ascending  degeneration  in  the  fibres  affected  in  the  first  process.  It 
is  important  to  recognise  this  complexity  of  mechanism. 

In  cases  of  rapid  course,  products  of  degeneration,  masses  of  myelin 
and  granule  corpuscles,  may  be  found  in  the  affected  parts  when  these 
are  examined  by  appropriate  methods. 

In  rare  cases  other  lesions  are  found  in  the  cord,  corresponding 
to  the  complications  already  described.  The  lateral  columns  may  be 
sclerosed  apart  from  any  thickening  of  the  membranes.  Diffuse 
myelitis  may  be  met  with,  and,  occasionally,  characteristic  insular 
sclerosis  has  been  found  in  the  cervical  region. 

The  posterior  nerve-roots  may  appear  normal  in  slight  cases:  it 
must  be  remembered  that  only  a  considerable  degree  of  alteration  can 
be  detected  even  by  the  microscope.  Often  their  disease  is  con- 
spicuous ;  when  the  cord  is  much  affected  they  are  invariably  affected, 
and  may  be  atrophied,  grey,  and  thin  to  the  naked  eye,  while  the 
microscope  shows  wasting  of  the  nerve-fibres  and  slight  increase  of 
connective  tissue.  The  intra-spinal  part  may  be  much  more  altered 
than  the  extra-spinal.  The  changes  extend  to  the  ganglia.  These 
structures  are  sometimes  normal,  occasionally,  however,  much  altered, 
and  the  mixed  nerve  immediately  beyond  them  may  be  almost  free  from 
*  Stroebe,  '  Cent.  f.  Allg.  Path.  u.  Path.  Anat.,'  1894,  Bd.  v. 


LOCOMOTOR   ATAXY. 


473 


degeneration.  The  anterior  nerve-roots  are  altogether  normal,  except  in 
the  rare  cases  in  which  the  anterior  cornua  have  suffered,  and  then  some 
of  their  fibres  may  be  degenerated,  and  the  atrophied  fibres  may  be 
traced  also  in  the  mixed  nerve-trunk  beyond  the  junction  of  the  roots. 
The  peripheral  spinal  nerves  have  been  found  degenerated  and  in 
various  degrees  of  atrophy  in  a  large  number  of  cases  of  tabes.*  The 
change  consists  in  a  -wasting  of  the  nerve-fibres,  beginning  in  the 
white  substance,  which  may  be  reduced  to  a  very  narrow  layer. 
Ultimately  the  axis-cylinders  pensh ;  they  are  said  to  present  ii-re- 
gular  swellings  when  the  sheath  begins  to  waste. f     There  may  be  a 


B 


slight  increase  in  the  interstitial  tissue  and 
nuclei,  but  the  change  commences  in  the 
nerve-fibres  themselves.  The  degeneration 
is  greatest  in  the  terminal  nerve-filaments, 
and  lessens  in  degree  as  the  nerves  are  exa- 
mined higher  up,  gradually  ceasing  in  the 
larger  trunks,  and  before  the  plexuses  or 
corresponding  main  divisions  are  reached, 
the  fibres  are  almost  always  healthy.  The 
sensory  fibres  seem  to  be  exclusively  affected. 
The  lesion  has  hitherto  been  found  chiefly  in 
the  sensory  nerves  that  supply  the  skin  and 
joints,  but  that  the  sensory  nerves  of  muscle 
undergo  a  similar  change  has  been  ascer- 
tained by  Dejerine.  It  is  probable  that 
these  afferent  muscle  nerves  are  invariably 
diseased,  especially  the  fibres  concerned 
in  conducting  the  impressions  that  guide 
co-ordination,  spinal  and  cerebellar. — on  the 
former  of  which  the  knee-jerk  depend  •■,  ac- 
cording to  the  theory  maintained  on  an  earlier 
page.  The  detection  of  this  degeneration, 
among  the  unchanged  motor  fibres  of  the 
nerves,  is  very  difficult,  and  this  explains  the 
paucity  of  evidence  of  it.  The  peripheral 
■degeneration  is  found  most  commonly  in  the 
legs,  but  is  met  with  also  in  the  arms  when 
these  are  the  seat  of  symptoms.  The  change 
in  the  nerves  bears  no  relation  to  that  in  the 
spinal  cord  in  degree,  or  even  in  existence, 
for  extreme  alterations  have  been  found  in  the  nerves  when  the  cord 
was  quite  normal.  On  the  other  hand,  in  some  cases,  the  nerves  have 
been  healthy. 

*  The  first  observation  was  made  by  Westphal,  but  the  extent  and  significance  of 
the  change  in  the  nerves  was  pointed  out  by  Pierret  (1880),  and  his  observation  was 
soon  after  confirmed  and  extended  by  Dejerine  and  Pitres. 

t  Schmaus,  *  Deut.  Arch.  f.  klin.  Med. '  1890. 


Pia.  130. —  Osseous  lesions 
in  tabes.  (After  Charcot.) 
A.  Atrophy  of  the  beiid 
of  the  femur.  B.  Exces- 
sive formation  of  callus 
after  so-called  "sponta- 
neous "  fracture  of  the 
ulna  and  radius. 


474  SPINAL    CORD. 

The  optic  nerves,  when  atrophied,  present  wasting  of  the  nerve- 
fibres,  and  nsually  a  remarkable  increase  in  the  interstitial  connective 
tissue,  which  constitutes  thick  tracts  of  gelatinous  aspect.  Dege- 
neration of  the  ascending  or  descending  roots  of  the  fifth  nerve  has 
been  repeatedly  found  (first  by  Westphal  in  1864)  in  cases  in  which 
symptoms  were  present  in  the  area  of  its  distribution.  Similar 
changes  in  the  roots  or  nuclei  of  other  cranial  nerves  have  also  been 
found. 

Pierret  has  described,  as  common  in  cases  with  vaso-motor  and 
visceral  symptoms,  a  degeneration  of  the  intermedio-lateral  tract  and 
adjacent  nerve-fibres  in  the  upper  part  of  the  cord,  traceable  up  the 
medulla  oblongata  in  the  region  of  the  so-called  "  slender  column," 
adjacent  to  the  accessory,  glosso-pharyngeal,  and  pneumogastric 
nuclei,  from  which  it  is  supposed  that  the  chief  visceral  and  vaso- 
motor influences  are  exerted.  The  sympathetic  has  been  examined  in 
several  cases,  and  found  healthy,  with  one  exception,  in  which  the 
sympathetic  on  the  left  side  of  the  neck  was  degenerated  in  associa- 
tion with  left  exophthalmos. 

In  the  diseased  bones  or  joints  conspicuous  changes  are  foxuid 
after  death.  There  is  occasionally  an  extraordinary  wasting  of  the 
articular  ends  of  the  bones,  which  seems  to  begin  by  rapid  erosion  of 
the  cartilages,  extending  quickly  to  the  bone  beyond,  the  extremity  of 
which  may  be  much  reduced  in  size  and  altered  in  form.  In  the  hip- 
joint,  for  instance,  in  a  case  recorded  by  Charcot,  the  edges  of  the 
acetabulum  had  been  removed,  and  the  whole  of  the  head  of  the  femur 
and  most  of  the  trochanter  had  disappeared  (Fig.  130). 

Similar  changes  have  been  found  in  most  of  the  larger  joints.  In 
the  bones  the  compact  tissue  has  been  found  thinner  and  m.ore  porous 
than  normal.  At  the  seat  of  old  fractures  a  large  amount  of  bony 
callus  is  formed  (Fig.  130),  and  sometimes  new  bone  is  found  in  the 
capsule  of  joints  that  have  been  injured. 

PATHOLoaT. — The  great  fact  of  the  pathology  of  tabes  is  that  it  ia 
a  neural  degeneration  in  the  sensory  nervous  system,  peripheral  and 
central.  In  both  peripheral  nerves  and  spinal  cord,  the  incidence  of 
the  disease  is  almost  exclusively  on  the  structures  concerned  in  afferent 
conduction.  The  cerebral  system  suffers  in  far  less  degree  than  the 
spinal,  and  in  more  irregular  manner,  but  in  the  occasional  peripheral 
degeneration  of  the  optic  nerve,  and  the  central  changes  in  the  roots 
of  the  fifth,  we  may  trace  the  same  law  of  distribution.  It  is  less 
dominant,  however,  than  in  the  spinal  system,  since  some  of  the 
cranial  nerve  symptoms  are  exclusively  motor.  The  motor  character 
of  the  leading  symptom,  ataxy,  is,  as  we  shall  see,  only  an  apparent 
and  not  a  real  exception  to  the  general  sensory  character  of  the 
malady. 

The  degeneration  commences  in  the  nerve-elements  themselves ; 
the  overgrowth  of  connective  tissue,  which  gives  to  the  lesion  its 
obtrusive   character,  is  secondary.     This  fact  has  been  observed  in 


LOCOMOTOR  ATAXY.  475 

the  peripTi'^ral  nerves  ;  as  regards  tlie  spinal  cord  it  rests  on  inference 
scarcely  less  certain.  Tliat  it  is  true  of  the  spinal  cord  has,  indeed, 
been  doubted,  chiefly  on  the  ground  of  the  thickening  of  the  walls  of 
the  vessels  seen  in  some  cases,  and  the  manner  in  which  the  increase  of 
tissue  seems  to  start  from  them.  But  the  "  system  "  character  of  a 
disease,  the  limitation  of  a  wide-spread  lesion  in  its  early  stage  or  slight 
degree  to  structures  having  a  common  function,  is  probably  in  all 
cases  proof  of  its  neural  origin,  i.  e.  its  origin  in  the  nerve-elements 
themselves.  In  tabes  the  disease  of  the  peripheral  nerves  depends  on 
decay  of  the  fibres,  and  the  partial  character  of  the  sensory  loss 
indicates  the  limitation  of  the  chief  lesion  to  fibres  of  one  sensory 
function.  There  is  every  gradation,  moreover,  between  the  cases  in 
which  the  interstitial  and  perivascular  disease  is  considerable,  and 
those  in  which  it  is  so  slight  that  there  is  nothing  to  suggest  a  primary 
interstitial  process  The  change  in  the  vessels  may  reach  a  high  degree 
in  the  posterior  median  columns  when  their  degeneration  is  purely 
secondary,  and  certainly  commences  in  the  nerve-fibres,  being  the 
result  of  a  focal  lesion ;  a  similar  thickening  of  the  vessels  has  also 
been  met  with  in  secondary  degenerations  in  other  parts.  It  is  most 
important  to  remember  that  the  overgrowth  of  the  neuroglia  is  a 
pathological  process  of  growth  disting-uishable  (and  to  a  large  extent 
distinct)  from  the  degeneration  of  the  nerve-fibres  that  excites  it.  It 
may  vary  in  degree  in  difl;erent  cases,  and,  even  in  the  secondary 
degeneration  of  peripheral  nerves,  may  assume  an  independent  energy 
and  even  present  an  inflammatory  character.  Once  excited,  the  process 
of  tissue-growth  may  be  largely  independent  of  its  cause.  Secondary 
vascular  disturbance  may  attend  it,  and  thus  a  sub-inflammatory  con- 
dition (or  even  a  true  inflammatory  condition)  is  probably  sometimes 
developed ;  it  may  invade  the  pia  mater,  and  through  this  may  spread 
widely. 

The  chief  causal  fact  regarding  the  disease  is  its  common  relation 
to  syphilis.  Tabes  is  generally,  although  possibly  not  invariably,  a 
post- syphilitic  disease ;  certain  elements  of  the  nervous  system 
degenerate  in  consequence  of  the  influence  on  them  of  previous 
syphilis.  There  may  be  indeed,  present  and  active  syphilis,  but, 
generally  the  active  stage  of  the  disease  is  over.  Syphilis  is  often  a 
prolonged  malady,  in  which  the  results  of  its  earlier  activity  sometimes 
coincide  with  its  later  specific  effect.  The  elements  that  suffer  have 
some  special  liability  to  degeneration,  their  nutrition  may  fail  from 
other  causes,  but  they  seem  to  possess  a  special  susceptibility  to  the 
influence  of  syphilis.  The  usual  symmetry  of  tabetic  lesions  indicates 
that  the  mechanism  by  which  syphilis  affects  the  nervous  system  is  a 
blood-state.  The  manner  in  which  the  peripheral  nerves  suffer  in 
many  cases,  brings  tabes,  in  spite  of  its  chronicity,  into  analogy  with 
the  more  acute  forms  of  peripheral  neuritis,  such  as  are  due  to  toxic 
agents,  sometimes  the  result  of  acute  specific  diseases.  The  long 
interval  which  elapses  between  gyphilis  and  tabes  and  the  common 


476  SPINAL  CORD. 

slowness  of  the  tabetic  process  are  only  in  proportion  to  the 
ch-ronicity  of  the  causal  malady,  compared  with  the  acute  specific 
diseases  tha,t  have  neuritic  sequelae  Further,  the  considerations 
mentioned  in  the  account  of  multiple  neuritis,  which  suggest  that  its 
immediate  cause,  when  it  is  due  to  an  acute  blood  disease,  is  a  product 
of  the  growth  of  the  organisms  of  the  primary  malady,  rather  than 
the  organisms  themselves,  hold  good  also  in  the  case  of  syphilis  and 
tabes.  The  degenerative  changes  in  the  nervous  system  differ  from 
the  lesions  of  active  syphilis,  both  in  character  and  in  time,  in  such  a 
way  as  to  make  the  assumption  reasonable  that  they  depend  upon 
some  product  of  the  growth  of  the  syphilitic  organisms,  a  product 
which  is  probably  a  chemical  toxine,  a  suggestion  first  made  by 
Striimpell,  and  widely  held  regarding  other  analogous  maladies.  Recent 
research  is  bringing  into  ever  greater  prominence  the  important  part 
played  by  such  products  in  the  generation  and  course  of  diseases.  In 
the  specific  diseases  that  have  these  sequelae,  there  may  be  more  than 
one  such  product  of  their  organisms,  causing  more  than  one  kind  of 
consequence.  The  differences  between  such  nerve  degenerations  as 
those  of  tabes,  and  other  late  effects  of  syphilis,  is  at  least  not  difficult 
to  comprehend  on  this  hypothesis ;  neither  is  the  fact — which  has 
been  so  great  a  difficulty  to  many — that  the  treatment  which  has  so 
speedy  an  effect  on  sjphilis  itself,  is  generally  without  influence  on  the 
degenerative  processes  that  sometimes  follow  it. 

Tabes  is  probably  a  rare  sequel,  when  regarded  from  the  side  of 
syphihs.  But  the  occurrence  of  such  consequences  in  some  instances 
and  not  in  others  has  analogies  in  the  case  of  almost  all  the  acute 
specific  diseases  that  have  similar  sequelae,  and  must  be  taken  as 
evidence  of  variations  in  the  precise  character  of  the  primary  disease. 
The  virus  of  syphilis  is  doubtless  organismal ;  this  has  not,  indeed, 
been  demonstrated,  but  the  analogy  it  presents  with  other  specific 
diseases  precludes  doubt  on  the  subject.  Similar  variations  in  the 
sequelae  are  familiar  to  us  in  the  case  of  other  diseases.  They  some- 
times have,  and  at  other  times  have  not,  consecutive  effects  upon  the 
nervous  system,  as  in  the  familiar  instance  of  diphtheria.  Diph- 
theritic paralysis  is  a  result  of  the  disease,  most  irregular  in  occurrence 
and  degree.  But  it  deserves  note  that  its  dependence  on  a  chemical 
toxin,  the  indirect  result  of  the  organisms,  has  been  conclusively 
proved  by  Sidney  Martin.  The  significance  of  the  fact  is  the  greater, 
inasmuch  as  its  effects  sometimes  closely  resemble  the  symptoms  of 
tabes. 

These  considerations  receive  a  notable'  emphasis  from  the  occur- 
rence of  tabes  in  children  who  are  the  subjects  of  inhei'ited  syphilis, 
to  whom  the  disease  in  early  Ufe  is  probably  confined.  It  has  been 
present  in  all  the  cases  that  have  come  under  my  notice.  But  the 
sequel  differs  much  from  those  that  are  related  to  other  diseases. 
Its  occurrence  is  quite  independent  of  the  thoroughness  of  treatment 
of  the  primary  disease. 


LOCOMOTOR  ATAXY.  477 

Tlie  pathological  facts  already  known  enable  us  to  understand  many 
of  the  symptoms  of  the  disease.  There  may  be  an  interruption  of  the 
sensory  path  in  one  or  both  of  two  places,  in  the  peripheral  nerves, 
and  in  the  posterior  root-fibres  as  they  enter  the  cord,  and  an  inter- 
ruption of  the  fibres  in  either  place  will  explain  the  loss  of  sensibility 
which  is  so  frequently  present.  The  pains  may  most  reasonably  be 
ascribed  to  the  molecular  changes  in  the  peripheral  nerve-fibres.  The 
intensity  of  a  sensation  is  no  measure  of  that  of  the  process  that  causes 
it.  Whether  the  affection  of  the  cells  of  the  posterior  ganglia,  or  of 
the  posterior  cornua  of  the  cord  *  takes  part  in  the  production  of  the 
symptoms  we  cannot  tell.  These  cells  are  the  nutritiona'l  centres  of 
the  sensory  "  neurons,"  within  the  influence  of  which  are  the  struc- 
tures that  degenerate.  It  is  noteworthy  that  the  affection  of  the 
peripheral  nerves  must  involve  the  structures  by  which  the  nerve- 
impulses  of  sensation  are  normally  generated  by  mechanical  and 
other  processes.  The  long  persistence  of  pains  without  any  increase 
in  the  symptoms,  shows  that  they  may  be  due  to  the  action  of 
structures  that  are  changed,  but  not  changing.  The  loss  of  reflex 
action  from  the  skin  is  explaiaed  by  the  interruption  of  the  sensory- 
path,  and  an  increased  irritability  of  the  sensory  nerves,  the  result  of 
even  slight  degenerative  changes,  sufiiciently  explains  both  the  hyper- 
sesthesia  and  the  increase  of  reflex  action  sometimes  observed. 

Whatever  theory  of  the  nature  of  the  so-called  "tendon-reflex" 
action  is  held,  the  loss  of  the  knee-jerk  must  be  explained  by  an 
interruption  of  the  sensory  path.  On  the  theory  I  have  advanced, 
the  arrest  of  impressions  from  the  afferent  muscular  nerves  abolishes 
the  muscle-reflex  action  on  which  the  local  irritability  depends  (see 
p.  25).  Many  other  facts  of  disease  show  that  this  irritability  is 
easily  lost,  and  that  a  very  slight  change  in  any  part  of  the  reflex 
arc,  too  slight  to  cause  other  symptoms,  is  sufiicient  to  arrest  the  knee- 
jerk. f  Hence  we  can  understand  that  this  loss  should  be  constant 
and  early.  It  is  not  improbable,  moreover,  that  these  nerves  suffer  in 
special  degree.  The  muscles  may  be  insensitive  to  pain,  e.  g.  that  of 
electrical  stimulation  Pressure  and  extension,  which  in  health  are 
painful,  sometimes  cause  no  sensation,  even  when  the  sMn  is  sen- 
sitive. 

The  mechanism  of  the  muscular  inco-ordination,  which  is  the 
obtrusive  symptom  of  the  disease,  has  been  the  subject  of  much  dis- 
cussion. Two  fundamental  facts,  however,  limit  the  problem.  First, 
the  ataxy  cannot  be  primarily  due  to  the  loss  of  cutaneous  sensibility. 

*  In  disease  of  the  nerve-roots  of  the  cauda  equina  there  is  always  ascending 
degeneration  of  the  posterior  median  columns,  but  not  of  the  antero-lateral  ascend- 
ing tract.  Hence  the  root-fibres  from  which  the  path  is  continued  by  this  tract 
must  end  in  nerve-cells  in  the  posterior  cornua,  and  the  degeneration  of  this  tract 
in  tabes  is  proof  of  the  degeneration  of  these  sensory  cells.  See  the  '  Lancet,' 
June  19th,  1886. 

+  lE-ff-  i^s  loss  after  diphtheria  when  there  are  no  other  symptomi. 


478  SPINAL    CORD. 

Disease  of  tlie  conducting  path  in  tlie  cord  may  cause  absolute 
anaesthesia  of  the  skin  without  the  least  ataxy.  Although  this  does 
not  prove  that  interruption  of  the  sensory  path  in  the  nerves,  between 
the  skin  and  the  reflex  centres,  may  not  cause  inco-ordination,  this 
element  seems  to  be  excluded  by  the  fact  that  there  is  no  relation 
between  the  ataxy  and  the  loss  of  feeling  in  the  skin.  There  may  be, 
in  tabes,  much,  ataxy  without  any  cutaneous  anaesthesia,  and  vice 
versa.  The  second  fact  is  that  ataxy  may  exist  in  considerable  degree 
when  the  lesion  is  solely  one  of  the  peripheral  nerves,  or  nerve-roots, 
and  the  posterior  columns  of  the  cord  are  free  from  disease.*  These 
two  facts,  taken  together,  seem  to  show  that  the  ataxy  may  be  pro- 
duced by  one  mechanism,  even  operating  alone,  the  disease  of  the 
afferent  muscle-nerves.  If  the  loss  of  the  knee-jerk  in  tabes  is  to 
be  taken,  as  I  believe  it  may,  as  an  indication  of  the  disease  of  these 
nerves,  the  constancy  of  the  loss  shows  the  constancy  of  the  presence 
of  this  element,  in  some  degree.  As  just  stated,  a  very  slight  degree 
of  disease  may  abolish  the  knee-jerk ;  it  is  probable  that  a  greater 
degree  is  needed  to  cause  inco-ordination,  and  a  still  greater  change  to 
produce  actual  insensibility  to  pressure  or  extension. 

In  unilateral  lesions  of  the  spinal  cord,  as  we  have  already  seen 
(p.  272) ,  there  may  be  loss  of  the  sense  of  posture,  with  intact  cutaneous 
sensibility,  on  the  side  of  the  lesion,  and  no  loss  of  this  sense  on  the 
opposite  side,  on  which  cutaneous  sensibility  is  lost.  In  such  a  case 
marked  ataxy  has  been  observed  on  the  side  on  which  the  sense  of 
posture  was  lost,  when  motor  power  returned.f  Whatever  effect  in 
causing  ataxy  is  produced  by  disease  of  the  path  of  muscular  sensi- 
bility in  the  spinal  cord  must  also  be  produced  by  interruption  of  the 
path  between  the  muscles  and  the  cord.  The  latter  must  also  arrest 
whatever  reflex  action  depends  upon  these  musclG-nerves,J  and  it  is 
possible  that  such  reflex  action  takes  some  part  in  the  mechanism  of 
muscular  co-ordination. 

In  this  connection  it  is  important  to  note  that  the  fibres  that  pass 
up  by  the  posterior  median  columns  seem  to  constitute  the  path  from 
these  nerves.    The  root-fibres  to  the  columns  pass  up,  as  the  path  is 

•  Apart  from  the  evidence  of  this  from  cases  of  characteristic  tabes,  a  very 
iastructive  case  has  been  recorded  by  Dr.  Hughes  Bennett,  in  which  all  the  sym- 
ptoms of  tabes  were  present  in  a  case  of  multiple  tumours  of  the  posterior  nerve- 
roots  ('  Clinical  See.  Transactions,*  vol.  xviii).  Ataxy  has  also  resulted  from  other 
processes,  such  as  injury,  that  have  caused  extensive  damage  to  the  posterior  roots 
alone. 

t  Gilbert;  see  foot-note,  p.  272. 

Ij:  In  a  case  in  wbich  the  incoordination  was  much  greater  in  the  right  leg  than 
in  the  left,  and  cutaneous  sensibility  was  equally  impaired  in  the  two,  a  strong  trac- 
tion on  the  calf  muscles,  by  forcible  passive  flexion  of  the  foot,  produced  a  distinct 
sensation  in  the  extended  muscles  of  the  left  leg,  and  no  sensation  in  those  of  the 
right.  In  another  ciise,  in  which  the  skin  was  sensitive  to  the  slightest  touch,  the 
patient  was  unconscious  of  a  vigorous  extension  of  the  toes  produced  by  faradio 
stimulation  of  thtir  sliort  extensor. 


LOCOMOTOR  ATAXY.  479 

proved  to  do,  without  decussating,  and  tliej  may  be  diseased  in  intense 
degree  when  there  is  no  loss  of  cutaneous  sensibility.*  This  tract 
is  affected  in  almost  all  cases  of  tabes  in  which  there  is  a  lesion  in 
the  cord,  and  in  all  such  cases  in  which  there  is  ataxy.  This  fact 
suggests  that  the  lesion  is  first  and  chiefly  of  the  nerye-fibres  from  the 
muscles,  t 

The  posterior  median  columns  (extensively  connected  with  the  cere- 
bellum) and  the  direct  cerebellar  tract,  probably  conduct  to  the  cere- 
bellum the  impressions  from  the  sensory  muscle-nerves.  If  so,  the 
disease  of  these  tracts  and  nerves  must  interfere  with  the  co-ordinating 
action  of  the  cerebellum,  and  cause  some  part  of  the  loss  of  function 
which  results  from  disease  of  the  cerebellum  itself. 

There  is  another  mechanism  which  may  co-operate  in  increasing 
ataxy.  The  vertical  fibres  of  the  postero-external  column  have  only 
a  short  course,  and  some  probably  connect  the  posterior  grey  matter 
at  adjacent  levels.  The  affection  of  these  fibres  seems  to  be  j)roved  by 
the  fact  that  the  comma-shaped  bundle  of  fibres  in  the  front  of  the 
postero-external  column  may  be  specially  degenerated.  This  lesion 
may  impair  the  association  of  the  sensory  structures,  and  so  cause  a 
want  of  harmony  in  the  central  mechanism.  The  theory  that  the 
ataxy  was  due  to  such  a  mechanism  was  advanced  by  Todd  in  1847. 
It  is  not  probable  that,  if  effective,  it  has  more  than  an  intensifying 
influence,  since  ataxy  may  exist  when  the  posterior  columns  are 
throughout  unaffected.  The  discovery  that  ataxy  may  result  from 
alcoholic  peripheral  neuritis  confirms  the  view  of  its  origin  above 
stated. 

Although  cutaneous  ansesthesia  cannot  alone  produce  ataxy,  it  may 
reasonably  be  assumed  to  increase  that  which  already  exists.  Sensa- 
tions from  the  skin  furnish  important  guidance  to  the  motor  cerebral 
centres,  and  are  probably  also  concerned  in  such  reflex  muscular  actions 
as  that  of  standing.  Similar  guidance  to  the  cerebral  co-ordinating 
centres  is  also  afforded  by  visual  impressions,  the  loss  of  which  distinctly 
augments  the  defect  of  co-ordination.  The  varying  characters  oi  the 
ataxy  in  different  cases  probably  depend  on  the  local  variations  in  the 
degree  of  change  in  the  muscle-nerves.  The  special  effect  of  the 
di  sease  of  other  elements  of  the  cord,  related  to  the  posterior  roots 
(as  the  posterior  vesicular  column,  Clarke's  column),  has  still  to  be 
ascertained. 

It  is  probable  that  co-ordination  is  chiefly  an  automatic  process 
depending  partly  on  muscle-reflex  actions,  and  on  the  connection  of 
neighbouring  sensory  structures  in  the  spinal  cord,  but  chiefly  on  the 
func\bn  of  the  cerebellum,  determined  by  the  connection  of  the 
*  This  posterior  tract  is  most  diseased  on  the  side  opposite  to  that  on  which  there 
is  most  degeneration  of  the  antero-lateral  ascending  tract. 

t  The  opinion  that  the  ataxy  depends  on  impairment  of  the  muscular  sense  was 
put  forward  in  1855  by  Dr.  Russell  Reynolds  j  see  p.  445.  Dejerine  has  also  asso- 
ciated the  ataxy  with  the  degeneration  of  the  peripheral  sensory  muscle-nerves 
(•  Archives  de  Physiologic,*  8rd  series,  vol.  iii,  p.  231). 


480  SPINAL   CORD. 

muscles  with  it,  and  that  the  interruption  of  this  connection  is  the 
chief  element  in.  the  inco-ordination  of  locomotor  ataxy.  It  is  probable 
also  that  the  automatic  processes  are  in  part  under  cerebral  control, 
guided  bj  sensory  impressions  which  do  not  enter  the  sphere  of 
consciousness,  and  that  the  derangement  of  this  control  will  intensify 
inco-ordination,  though  incapable  of  producing  it.  We  do  not  at 
present  know  to  what  extent,  in  any  given  case,  the  symptoms  are  due 
to  the  cord  disease  or  to  the  peripheral  nerve  lesions.  Apparently 
pains,  ataxy,  and  anaesthesia  may  be  due  to  either.  The  question  can 
only  be  decided  by  the  comparison  of  symptoms  and  pathological 
changes  iu  a  large  number  of  cases.  That  anaesthesia  may  be  due  to 
the  peripheral  changes  is  proved  by  the  observed  correspondence  of  the 
two  in  distribution  (Dejeriue). 

The  trophic  changes  in  the  skin,  bones,  and  joints  are  probably  due 
to  the  process  of  degeneration  in  the  peripheral  nerves.  The  degenera- 
tion has  been  found  iu  all  cases  of  the  kind  in  which  it  has  been  looked 
for,  and  found  also  in  the  nerves  of  diseased  joints.  The  pathology  of 
the  muscular  wasting  has  been  already  mentioned. 

The  pains  in  the  region  of  the  fifth  nerve  are  explained  by  the  lesions 
in  it  root,  especially  in  its  ascending  root,  which,  coming  up  from  the 
medulla,  is  homologous  with  the  posterior  spinal  roots.  This  nerve, 
indeed,  as  Pierrot  has  pointed  out,  represents  the  sensoiy  roots  of 
almost  all  the  motor  cranial  nerves.  The  degeneration  of  the  optic 
nerve  is  fairly  comparable  with  that  of  the  peripheral  spinal  nerves. 
Considering  the  special  character  of  the  optic  nerve,  the  absence  of 
degeneration  of  the  retina  does  not  seem  to  destroy  the  analogy 
between  the  two.  Eegarding  the  pathology  of  the  visceral  crises  we 
know  but  little. 

The  transient  motor  symptoms  in  the  limbs,  and  in  the  eyeball 
muscles,  are  apparently  of  functional  origin.  They  must  be  distin- 
guished from  the  lasting  palsies,  which  are  probably  due  to  degene- 
rative processes  in  the  nerves  or  nuclei.  Such  transient  paralysis 
may  precede  enduring  paralysis  due  to  nuclear  degeneration.  The 
loss  of  function  of  the  internal  ocular  muscles,  being  persistent,  must 
be  ascribed  to  degeneration  of  the  related  centres.  But  even  the 
hght-reflex  may  return  ^^in  rare  cases)  after  it  has  been  absent  for 
years,  and  its  loss  must  therefore  depend,  at  least  sometimes,  on 
molecular  changes  capable  of-  recovery.  It  is,  indeed,  important  to 
remember  that  tabes  is  a  disease  which  begins  as  a  derangement  of 
molecular  nutrition,  which  is  probably  only  to  be  discerned  when 
relatively  great  in  its  degree.  If  we  also  remember  that  this  derange- 
ment is  the  result  of  some  toxic  material  or  virus  circulating  in  the 
blood,  we  can  understand  better  the  leading  facts  of  the  disease,  and 
especially  the  variations  ia  its  course,  its  degree,  and  the  precise 
character  of  the  symptoms. 

Diagnosis. — The  diagnosis  of  tabes  rests  on  the  combination  of 
symptoms  already  described.    In  the  early  stage,  the  loss  of  the  knee* 


LOCOMOTOR  ATAXY  481 

jert,  together  witli  pains,  or  unsteadiness  on  standing  with  the  feet 
together  and  the  eyes  closed,  justifies  a  diagnosis  of  the  commencing 
affection,  provided  we  can  exclude  toxic  peripheral  neuritis  (especially 
that  due  to  alcohol),  diabetes,  and  diphtheritic  palsy.  A  lesion  of  the 
anterior  cornua  or  nerve-roots  is  excluded  by  the  absence  of  wasting  of 
the  muscles  or  change  in  their  irritability.  The  diagnostic  value  of 
the  loss  of  the  knee-jerk  can  hardly  be  overrated.  It  is  probably  never 
absent  in  health.  If  there  is  doubt  as  to  its  loss,  the  precautions 
recommended  on  p.  21  should  be  adopted.  When  it  is  lost,  and  reflex 
action  is  in  excess,  a  true  reflex  movement  may  sometimes  simulate 
the  jerk ;  the  distinction  is  that  the  tap  sometimes  causes  a  movement 
and  sometimes  does  not;  that  an  interval,  brief  but  appreciable, 
elapses  before  the  movement  occurs;  that  a  similar  movement  is 
caused  by  a  prick  on  the  skin,  and  that  the  delayed  contraction  some- 
times occurs  in  the  other  leg,  or  in  the  adductors  of  both  legs.*  In 
cases  in  which  the  knee-jerk  is  present,  the  diagnosis  of  tabes  is  only 
justified  by  distinct  and  characteristic  inco-ordination.  In  the  few 
cases  of  this  kind  that  have  come  under  my  own  observation,  the  knee- 
jerk  has  been  either  unequal  on  the  two  sides,  or  has  been  lost  on  one. 
Such  abnormality  is  probably  the  rule  in  these  cases,  and  the  diagnosis 
is  thus  facilitated.  In  a  case  with  lightning  pains,  but  neither  inco- 
ordination nor  loss  of  knee-jerk,  a  suspicion  of  tabes  would  be  justified 
by  the  presence  of  some  other  symptom,  such  as  retention  or  incon- 
tinence of  urine,  loss  of  sexual  power,  or  loss  of  the  iris-reflex.  As  we 
have  seen,  it  is  probable  that  cases  occur  in  which  these  pains  exist 
alone, — a  condition  that  may  be  termed  "  tabetic  neuralgia." 

The  loss  of  the  iris-reflex  is  of  great  diagnostic  importance.  It 
shows  that  a  degenerative  process  is  at  work  in  the  nervous  system, 
and  it  suggests,  therefore,  that  other  symptoms  are  also  due  to  de- 
generation. But  since  the  iris-reflex  is  not  always  lost  in  tabes,  the 
negative  significance  of  a  normal  reflex  is  far  less  than  is  the  positive 
significance  of  its  loss.  The  practical  value  of  this  symptom  can 
hardly  be  overrated.  It  puts  the  observer,  so  to  speak,  on  the  track 
of  nerve  degeneration.  Its  loss  is,  moreover,  so  often  due  to  preceding 
syphilis,  tha,t  it  should  always  suggest  this  antecedent. 

There  are  certain  diseases  with  which  tabes  is  especially  liable  to 
'be  confounded.  One  of  these  is  multiple  alcoholic  neuritis.  The 
ordinary  form  of  this  disease  is  readily  distinguished  by  the  symme- 
trical paralysis  which  is  its  chief  manifestation.  But  the  variety 
which  closely  resembles  locomotor  ataxy  is  the  "alcoholic  pseudo- 
tabes" (p.  159),  in  which  the  diagnosis  maybe  very  difiicult.  This 
is  not  surprising,  because  it  resembles  tabes  pathologically ;  the  lesion 
is  a  "  parenchymatous  neuritis,"  a  subacute  degeneration,  beginning 
in  the  nerve-fibres,  and  similar  to  the  peripheral  form  of  tabes.      It 

*  It  is  impossible  to  exaggerate  the  difBculty  presented  by  touie  [latientsin  ascer- 
taining whether  the  knee-jerk  is  or  is  not  lost,  and  the  care  needed.  Repeated 
observations  are  desirable  in  doubtful  cases. 

VOL.  I.  31 


482  SPINAL   COED. 

often  affects,  howeyer,  in  some  degree,  the  motor  as  well  as  the  sen- 
sorj  nerves,  and  there  is  then  some  weakness  in  the  distal  portions  of 
the  hmbs.  An  altered  electrical  reaction  may  be  found  in  the  muscles 
(never  in  the  weakness  sometimes  met  with  in  early  tabes),  and  there 
is  generally  marked  muscular  tenderness,  scarcely  ever  present  in 
tabes.  The  pains  have  not  the  "lightning"  character,  and  the 
sphincters  escape.  The  pupils  act  normally,  but  this  is  not  an  abso- 
lute distinction,  since  they  may  be  unaffected  in  tabes.  A  history  of 
alcoholism  may  help  the  diagnosis,  and  so  may  improvement  when 
alcohol  is  withdrawn.  By  attention  to  all  these  points,  a  diagnosis 
can  generally  be  made  without  much  difficulty. 

When  the  symptoms  and  lesion  of  tabes  are  combined  with  those 
of  general  paralysis  of  the  insane,  it  may  be  doubtful  in  which  cate- 
gory a  case  should  be  placed.  The  question  is  rather  one  of  the  pre- 
ponderance of  the  symptoms  of  one  or  the  other  malady  than  of 
absolute  distinction  between  them.  In  most  cases,  however,  in  which 
this  combination  exists,  the  symptoms  of  general  paralysis  become 
more  pronounced  as  time  goes  on,  and  the  spinal  symptoms,  which  at 
first  were  the  most  conspicuous,  pass  into  the  background. 

All  common  forms  of  paraplegia  are  distinguished  by  the  early 
loss  of  power,  and  by  the  persistence  of  the  knee-jerk,  often 
emphasised  by  its  excess  and  by  the  occurrence  of  a  clonus  in  the 
extended  rectus  or  calf  muscles.  If  weakness  supervenes  in  tabes,  it 
is  usually  late  in  the  course  of  the  disease,  and  the  other  symptoms 
have  been  and  are  well  marked.  If  the  knee-jerk  has  been  once 
lost  in  this  disease,  it  is  not  reproduced  by  secondary  lesions  of 
the  cord  which  ordinarily  increase  the  knee-jerk.*  The  same  distinc- 
tions usually  suffice  for  the  diagnosis  from  the  combined  form  of 
paralysis  and  inco-ordination  that  I  have  termed  "  ataxic  paraplegia." 
In  this  the  knee-jerk  is  excessive,  and  there  is  foot-clonus,  while  pains 
and  anaesthesia  are  absent ;  spasm  supersedes  ataxy.  In  other  forms 
of  combined  disease  of  the  cord  the  diagnosis  depends  chiefly  on  the 
recognition  of  union. 

Acute  lesions  of  the  cord,  probably  situated  in  the  postero-external 
cohunn,  may  produce  inco-ordination  and  pain,  but  the  suddenness  of 
the  onset,  the  limitation  of  the  symptoms,  and  their  tendency  to  sub- 
side, usually  suffice  for  the  distinction.  Symptoms  from  this  cause 
are  far  more  common  in  the  arms  than  in  the  legs.  Ataxy  in  one 
limb  may  also  be  produced  by  a  tumour  growing  in  the  posterior 
column,  but  other  symptoms  indicate  the  invasion  of  adjacent  struc- 
tures, as  was  conspicuous  in  the  case  figured  in  the  chapter  on 
tumours  of  the  cord.  All  these  cases  present  the  characteristics  of 
a  focal  lesion  with  random  consequences,  rather  than  of  a  system 
disease  witli  limitation  to  a  special  function. 

The  loss  of  perception  of  pain,  with  preserved  tactile  sensibility, 

*  See,  however,  an  exception  to  this,  referred  to  on  p.  455.  The  paper  referred 
to  mentions  oihers. 


LOCOMOTOR   ATAXY.  483 

when  it  involves  tlie  hands,  may  arouse  a  suspicion  of  syringomyelia. 
But  the  loss  of  the  knee-jerk  in  tahes,  and  the  absence  of  the  momen- 
tary pains  in  syringomyelia,  will  enable  a  correct  diagnosis  to  be  made 
€ven  without  corroborative  symptoms. 

In  diphtheritic  paralysis  occurring  long  after  the  sore  throat,  or  in 
which  the  nature  of  this  was  not  recognised,  the  loss  of  the  knee-jerk 
may  cause  tabes  to  be  suspected  ;  but  the  nature  of  the  case  is  almost 
always  rendered  clear  by  the  jaaralysis  of  accommodation  and  of  the 
palate  which  precedes  the  ataxy,  and  should  be  suggested  by  the 
r.bsence  of  pains  and  the  presence  of  weakness.  If  true  tabes  follows 
diphtheria,  with  lasting  ataxy,  it  is  so  rare  as  to  be  of  little  practical 
importance. 

When  the  dorsal  region  of  the  cord  is  chiefly  affected,  the  severity 
of  the  pains  in  the  trunk  may  lead  to  a  suspicion  of  disease  of  the 
vertebral  bones,  but  in  the  latter  the  pain  is  local  and  fixed,  and  this, 
with  the  characteristic  increase  of  pain  when  the  patient  moves,  should 
prevent  error.  It  is  still  more  frequent  to  mistake  such  cases  for 
neuralgia  of  the  intercostal  nerves,  but  even  if  there  are  no  pains  in 
the  legs  to  suggest  tabes,  the  knee-jerk  and  the  light-reflex  of  the  iris 
are  lost  in  tabes.  Experience  shows  that  the  most  frequent  errors  in 
diagnosis  are  due  to  a  mistake  as  to  the  nature  of  the  pains,  which 
are  mistaken  for  rheumatism,  especially  when  influenced  by  weather, 
or  for  sciatica  when  referred  to  the  region  of  the  sciatic  nerve,  or  for 
some  form  of  gout  if  the  disease  exists  in  the  patient  or  his  family,  or 
in  the  mind  of  the  physician.*  In  the  rare  cases  in  which  the  cranial 
nerves  are  disordered  and  pains  are  felt  in  the  head,  the  symptoms 
may  be  mistaken  for  trigeminal  neuralgia.  The  obscurity  of  the 
symptoms  may  be  increased  in  such  cases  by  a  sense  of  painful  con- 
striction in  the  face,  analogous  to  the  girdle-pain  felt  around  the 
trunk  (see  p.  453). 

The  only  organic  intra- cranial  disease  that  may  be  taken  for  tabes 
is  tumour  of  the  cerebellum.  In  some  cases  of  tabes  the  unsteadiness 
resembles  that  of  cerebellar  origin.  Loss  of  the  knee-jerk  is  strongly 
in  favour  of  tabes ;  in  cerebellar  tumour  increase  of  the  jerk  is  the 
rule,  and  loss  is  only  to  be  regarded  as  not  absolutely  incompatible. 
Anaesthesia  and  lightning  pains  are  conclusive  evidence  of  the  spinal 
disorder,  while  in  cerebellar  tumour  severe  occipital  headache  is  almost 
invariable,  and  considerable  optic  neuritis  is  extremely  common.  Optic 
nerve  atrophy  is  primary  in  tabes,  post-neuritic  in  tumour. 

The  various  visceral  crises  are  often  mistaken  for  primary  disorders 
of  the  deranged  organs.  Recurring  attacks  of  the  character  described 
should  always  arouse  suspicion  of  their  possibly  tabetic  nature,  and  an 
examination  of  the  knee-jerk  will  usually  decide  the  point. 

*  In  one  case,  in  which  tabetic  trunk-pain  was  chiefly  felt  at  the  level  of  the 
epigastrium,  a  physician  of  repute  not  only  made  a  diagnosis  of  gastric  gout,  but 
refused  to  modify  his  opinion  when  loss  of  the  knee-jerk  was  discovered,  and  the 
patient  became  unsteady  in  gait. 


484  SPINAL    COED. 

PKOaNOSis. — The  prognosis  in  tabes  corresponds  witli  tlie  facta 
stated  as  to  the  course  of  the  disease.  It  does  not,  as  was  once 
thought,  deserve  the  epithet  "  progreissive,"  given  to  it  before  the  pre- 
ataxic  stage  was  known  and  before  the  disease  had  been  widely 
observed.  Arrest  is  frequent,  and  considerable  improvement  is  not 
rare.  The  earlier  the  stage  of  the  disease  the  better  is  the  prognosis. 
In  the  first  stage  the  disease  often  becomes  stationary.  When  ataxy 
is  developed  the  prospect  of  arrest  is  less  than  in  the  first  stage,  but  is 
still  considerable.  Occasionally,  even  in  the  typical  form  of  the  disease, 
the  amount  of  improvement  is  very  great.  One  patient  was  scarcely 
able  to  walk  across  the  room,  in  consequence  of  the  inco-ordination, 
when  he  came  under  treatment,  and  at  the  end  of  six  months  his  gait 
was  scarcely  distinguishable  from  that  of  a  healthy  person.  When 
the  third  stage  is  reached  the  possibility  of  improvement  is  far  less, 
but  is  not  quite  absent.  A  woman  suffering  from  pure  tabes,  when 
admitted  to  hospital,  had  been  unable  even  to  stand  for  six  months,  on 
account  of  the  extreme  ataxy  ;  but  she  improved  so  much  that  in  a  few 
months  she  was  able  to  walk  about  the  room  without  help,  and  has  con- 
tinued, now  for  six  years,  to  do  her  household  work  without  diflSculty, 
and  even  to  walk  some  miles  with  the  aid  of  a  stick.  On  the  other  hand, 
in  some  cases  the  malady  increases  in  spite  of  every  effort,  and  even 
when  there  is  permanent  arrest  or  improvement  it  is  rare  for  all 
symptoms  to  disappear.  The  knee-jerk  generally  remains  absent,  and 
often  attacks  of  pain  continue  to  occur.  It  is  difficult  to  find  any 
trustworthy  indications  to  guide  the  prognosis  in  an  individual  case. 
The  fact  of  preceding  syphilis  has  little  influence  on  the  prognosis. 
Only  when  the  symptoms  have  developed  rapidly  and  the  syphilis  is 
recent  is  there  any  possibility  of  benefit  from  antisyphiHtic  treatment. 
In  all  cases,  however,  in  which  the  onset  of  symptoms  is  acute  there  i3 
more  prospect  that  they  will  pass  away  than  if  they  develop  slowly. 
In  most  cases  the  only  trustworthy  guide  to  prognosis  is  the  observed 
tendency  of  the  individual  case. 

Of  special  symptoms,  the  pains  are  often  the  most  distressing,  and 
unfortunately  they  are  the  most  obstinate  symptom  of  the  disease. 
They  may  persist  in  undiminished  severity  when  other  symptoms 
lessen ;  and,  on  the  other  hand,  they  may  become  trifling  when  the 
disease  increases.  Diminution  of  the  pain  is  a  good  sign  if  other 
symptoms  are  stationary,  but  not  if  these  increase.  It  is  often,  how- 
ever, a  solace  to  the  patient  to  know  that  persistence  of  the  pains  does 
not  mean  progress  in  the  disease.  The  optic  nerve  atrophy  is  usually 
progressive,  but  not  invariably,  as  is  often  asserted.  Occasionally  its 
progress  is  arrested,  and  more  frequently  when  it  is  greater  in  one 
eye  than  when  both  are  involved.  Strange  to  say,  the  occurrence  of 
optic  nerve  atrophy  makes  the  prognosis  as  to  the  spinal  symptoms 
better ;  in  most  cases  the  spinal  lesion  remains  in  the  first  stage. 

Treatment. — The  disease  is  one  in  which  treatment  often  has  a 
very  clear  influence,  not  only  in  relieving  suffering,  but  in  determining 


LOOOMO'l'OE   ATAXr.  485 

the  arrest  or  diminution  of  the  disease.  This  is  true  especially  of 
treatment  by  drugs.  But  the  first  care  should  be  to  secure  freedom, 
as  far  as  possible,  from  all  influences  capable  of  increasing  the  disease, 
and  from  whatever  is  likely  to  depress  the  nervous  system.  Excessive 
mental  work,  anxiety,  and  physical  fatigue  are  all  harmful.  In 
severe  and  acute  cases,  absolute  rest  for  a  week  or  two  is  often  useful 
ut  the  onset  of  treatment.  If  the  patient  walks  about,  exercise  should 
always  stop  short  of  fatigue.  Care  should  be  taken  to  avoid  the  risk 
■of  falls;  a  severe  concussion  of  the  spine  will  sometimes  excite  to 
fresh  activity  a  stationary  disease.  Exposure  to  cold  is  also  injurious ; 
a  severe  exposure  may  cause  acute  mischief.  If  practicable  it  is  well 
that  the  winter  should  be  spent  in  a  warm  and  dry  climate.  A  sea 
voyage  is  sometimes  useful,  combining,  as  it  does,  a  maximum  of 
fresh  air  with  a  minimum  of  exertion;  it  has,  however,  less  actual 
effect  than  might  be  expected.  If  there  is  much  ataxy,  the  risk  of 
falls  from  the  motion  of  the  ship  must  be  taken  into  consideration. 
It  is  the  most  useful  in  cases  that  are  already  stationary;  such 
patients  often  return  with  a  diminution  in  all  their  symptoms.  When 
the  disease  is  advancing,  a  voyage  often  does  harm  rather  than  good. 

The  digestive  organs  should  be  kept  in  careful  order;  an  attack 
of  indigestion  or  constipation  is  often  attended  by  lightning  pains, 
which  cease  when  an  aperient  has  acted.  Food  should  therefore 
be  light  in  quality,  and  easily  digestible.  The  change  from  an  active 
to  an  inactive  life,  which  tabes  often  causes,  may  lead  to  the  develop- 
ment of  gout  in  an  individual  predisposed  to  it,  either  by  inheritance 
or  by  his  previous  mode  of  life ;  and  the  tendency  must,  as  far  as 
possible,  be  obviated  by  regulation  of  diet  and  by  frequent  aperients. 
Smoking  should  be  either  avoided  or  limited  to  a  small  amount. 
Alcoholic  excess  is  especially  harmful ;  it  sometimes  excites  a  very 
severe  exacerbation  of  the  symptoms;  this  is  not  surprising,  since, 
as  we  have  seen,  chronic  alcoholism  may  cause  a  disease  resembling 
tabes  in  its  symptoms  and  pathology.  Sexual  excess  is  also  most 
injurious.  Many  patients  are  preserved  from  this  danger  by  the 
disease  itself,  but  it  is  not  always  so.  Excess  seems  to  have  a  special 
influence  on  the  optic  nerve  atrophy.  Several  very  painful  instances 
of  this  have  come  under  my  notice.  A  man  in  the  early  stage  of  tabes, 
with  slight  atrophy,  some  peripheral  limitation  of  the  fields,  but  little 
impairment  of  acuity  of  vision,  started  on  a  voyage  from  Austraha  to 
England.  The  day  before  he  started  he  married,  and  when  he  reached 
this  country  he  was  quite  blind. 

The  influence  of  drugs  on  the  disease  is  unquestionable  in  many- 
cases,  and  if  they  are  used  wisely,  the  more  a  physician  sees  of  the 
disease,  the  more  highly  is  he  likely  to  esteem  them.  There  is  no 
specific  for  the  malady,  and  in  some  cases  the  morbid  tendency  over- 
powers every  influence  that  can  be  brought  to  bear  upon  it.  Moreover, 
the  influence  of  drugs  is  variable ;  that  which  seems  to  do  much  good 
in  one  case  has  no  influence  on  another,  and  this  is  true  also  of  the 


486  SPINAL   COKD. 

same  case  at  diffei'ent  periods.  Hence  tlie  therapeutic  statements  that 
have  been  made  hj  various  observers  are  somewhat  discordant. 

The  first  question  is  the  influence  of  anti syphilitic  agents.  In 
tabes  such  influence  is  distinct  only  in  rare  cases,  chiefly  xmder  the 
conditions  mentioned  in  the  section  on  prognosis.  In  those  circum- 
stances full  doses  of  iodide  of  potassium  should  be  given,  and  if  the 
interval  since  the  primary  disease  is  short,  mercury  may  be  rubbed  in 
until  the  gums  are  slightly  affected.  In  cases  of  slow  development, 
several  years  after  primary  syphilis,  such  treatment  seldom,  does  good, 
and  sometimes  does  harm.  But  so  widely  spread  is  the  opinion  that 
every  consequence  of  syphilis  is  amenable  to  treatment,  that  it  is  often 
well  to  avoid  the  suspicion  of  neglect,  and  to  clear  the  therapeutic 
ground,  by  a  course  of  iodide  of  potassium  for  five  or  six  weeks. 
Yery  small  doses  of  mercury,  such  as  ^  grain  of  the  red  iodide,  may 
also  be  given  for  a  longer  time,  and  may  often  be  conveniently  com- 
bined with  other  drugs.  It  seems  sometimes  to  have  a  tonic  influ- 
ence in  these  cases.  But  in  such  late  cases,  and  in  all  the  late  nerve 
degenerations  that  follow  syphilis,  energetic  mercurial  treatment  only 
does  harm.  Its  depressing  influence  seems  to  increase  the  degenera- 
tive tendency,  and  to  hasten  the  progress  of  the  disease, — sometimes 
even  to  induce  its  extension. 

Of  drugs  that  have  been  recommended  for  tabes,  those  that  are 
most  useful  are  arsenic,  iron,  quinine,  aluminium,  nux  vomica  and 
strychnia,  nitrate  of  silver,  Calabar  bean,  belladonna,  ergot,  phos- 
phorus. Of  these  arsenic  is  certainly  that  which  most  frequently  does 
distinct  good.  Most  of  the  cases  in  which  I  have  known  the  greatest 
improvement  to  occur,  have  been  taking  it  at  the  time.  In  several  of 
these  cases  there  was  no  other  change  in  the  conditions  of  life  to 
which  the  improvement  could  be  ascribed.  The  form  in  which  arsenic 
is  given  matters  little ;  n^iij  to  TTl^vij  of  the  Liq.  Arsenicalis  may  be  given 
in  a  mixture,  or  -^^  gr.  of  arseniate  of  soda  in  a  pill.  Iodide  of  arsenic 
has  no  special  advantage,  and  is  not  so  well  borne.  This  or  any 
other  drug  must  be  continued  for  at  least  six  weeks  before  an  opinion 
of  its  influence  can  be  formed.  Eapid  improvement  can  never  be 
expected  in  so  chronic  a  disease.  But  it  is  not  well  to  continue  any 
drug  for  more  than  about  three  months  at  a  time.  Most  good  is 
effected  by  alternating  two  or  more ;  the  return  to  one  that  had  ceased 
to  do  good  is  often  beneficial.  In  connection  with  the  influence  of 
arseuic,  it  is  interesting  to  note  the  well-known  effect  of  the  drug  in 
improving  the  nutrition  of  the  skin,  and  the  frequency  with  which 
the  cutaneous  nerves  are  degenerated  in  tabes.  Quinine,  or  the 
extract  of  nux  vomica, -may  be  usefully  combined  with  arsenic;  or  a 
small  dose  of  mercury  may  also  be  given,  n\xv  of  Liq.  Hyd.  Bichlor. 
with  rr\Y  of  Liq.  Arsenicalis,  or  -^  gr.  of  the  red  iodide  with  -^V  g^"-  of 
arseniate  of  soda  in  a  pill.  Occasionally  arsenic  seems  to  irritate  the 
nerve-centres,  but  smaller  doses  may  then  be  borne,  and  may  be  useful. 
"Nitrate  of  silver  and  phosphorus  are  less  effective,  but  may    some- 


LOCOMOTOR    ATAXY.  487 

times  be  given  alternately  with  arsenic.  The  chloride  of  aluminium 
is  another  agent  that  I  have  found  of  distinct  service,  2 — 4  gr. 
twice  or  three  times  a  day.  It  certainly  diminishes  the  severity  of  the 
pains  and  the  tendency  to  them. 

Calabar  bean  has  been  recommended  as  producing  temporary  im- 
provement in  many  chronic  spinal  diseases.  Its  influence  in  tabes  is 
not  great.  Ergot  has  been  esteemed  highly  by  some,  but  is  seldom 
distinctly  effective.  Belladonna  is  sometimes  useful,  especiaUy  when 
there  is  a  tendency  to  incontinence  of  urine.  Strychnia  in  the  same 
cases  sometimes  produces  marked  improvement,  especially  combined 
with  a  small  dose  (^-^q  gr.)  of  nitro- glycerine,  which  causes  the  blood 
containing  the- tonic  to  pass  more  freely  to  the  nerve-centres. 

Counter-irritation  to  the  spine,  by  blisters  or  the  actual  cautery,  is 
often  useful,  chiefly  in  the  cases  just  mentioned,  in  which  the  dis- 
ease develops,  or  the  symptoms  increase,  rapidly,  or  in  which  there 
is  spinal  pain  or  tenderness.  It  is  not  probable  that  it  influences  the 
process  of  degeneration,  but  it  may  lessen  any  secondary  inflammation. 

Of  special  symptoms  that  require  treatment,  the  pains  are  the 
most  important.  Unfortunately  they  are  often  very  obstinate,  and 
their  variability  renders  it  difficult  to  estimate  the  influence  of  treat- 
ment upon  them.  Severe  paroxysms  may  yield  only  to  hypodermic 
injections  of  morphia,  but  this  should  be  reserved  for  the  most  intense 
attacks,  since  its  use  is  undesirable  for  a  recurring  symptom  which  may 
continue  for  years.  Indian  hemp  is  often  effective  ;  gr.  J  or  -^  should 
be  given  every  three  or  four  hours  during  an  attack.  But  the  most 
useful  agents  for  relieving  a  paroxysm  are  antipyrine  and  acetanilide. 
One  or  the  other  of  these  seldom  fails ;  should  they  do  so,  exalgin  or 
phenacetin  or  migranin  may  succeed.  Hypodermic  injections  of  cocaine 
generally  arrest,  for  several  hours,  pains  that  are  superficial  in  character, 
especially  if  accompanied  by  hypersesthesia  of  the  skin .  The  injec- 
tion should  be  of  -^  to  ^  of  a  grain,  at  the  upper  part  of  the  region 
to  which  the  pain  is  referred.  Cocaine  has  no  influence  over  the 
deepar  pains.  Superficial  pain  is  also  relieved  by  the  local  applica- 
tion of  chloroform  sprinkled  on  spongiopiline  (or  lint  and  oiled  silk). 
Occasionally  the  pains  are  lessened  by  a  warm  bath.  Counter-irritation 
to  the  spine,  as  by  repeated  sinapisms,  may  give  some  relief.  These 
measures,  however,  only  relieve  paroxysms,  and  do  not  prevent  recur- 
rence. Chloride  of  aluminium  is  of  considerable  service  in  lessening 
the  tendency  to  the  occurrence  of  the  pains.  Two  to  four  grains  may 
be  given  three  times  a  day.  It  is  readily  soluble,  and  may  be  given 
in  combination  with  other  drugs. 

Paroxysmal  visceral  symptoms  often  yield  to  antipyrine  or  aceta- 
nilide, but  when  severe,  morphia  alone  mitigates  their  intensity. 
Slight  gastric  crises  may  be  relieved  by  simultaneous  sinapisms  to  the 
epigastrium  and  neck.  Laryngeal  spasm  is  usually  at  once  relieved 
by  nitrite  of  amyl  or  nitro-glycerine ;  the  local  application  of  cocaine  is 
also  useful.      Weakness  of   the  bladder   is  sometimes  lessened  by 


488  SPINAL    COED. 

strjchnia  or  belladonna       Strychnia  may  be   given  by  hypodermic 
injection,  as  recommended  in  the  chapter  on  muscular  atrophy. 

Blisters  or  sores  on  the  feet  should  receive  careful  local  treatment 
until  they  are  well.  If  neglected  they  may  become  very  troublesome. 
The  patients  should  be  cautioned  never  to  cut  a  corn  ;  a  perforatiag 
ulcer  is  often  set  up  by  a  corn  being  cut  too  deeply.  The  epidermis 
should  be  softened  with  an  alkali  and  rubbed  off  with  pumice-stone. 

Attention  to  the  bladder  is  one  of  the  most  important  elements  in 
the  treatment  of  tabes,  as  in  that  of  all  diseases  of  the  spinal  cord, 
chronic  and  acute.  If  there  is  any  reason  to  believe  that  the  bladder 
is  imperfectly  emptied,  a  catheter  should  be  passed;  and  if  residual 
\irine  is  found,  the  bladder  should  be  emptied  perfectly  and  washed 
out  every  day  or  every  other  day.  !N"o  hesitation  need  be  felt  in  the 
use  of  the  catheter  in  these  cases,  provided  very  strict  antiseptic  pre- 
cautions are  taken.  Many  patients  die  every  year  from  want  of  the 
catheter,  because  it  is  thought  that  all  is  right  since  they  pass  urine 
freely.  The  residual  accumulation,  or  the  sUght  cystitis,  sets  up 
chi'onic  pyelo-nephritis,  which  develops  insidiously,  and  is  unsuspected 
until  mysterious  febrile  disturbance  occurs,  chronic  or  acute,  and  leads 
to  death.  It  is  probable  that  the  onset  of  the  final  acute  disturbance 
is  sometimes  determined  by  the  passage  of  a  catheter,  just  as  this  will 
cause  a  rigor  or  transient  hsematuria  in  a  healthy  person.  I  have  seen 
many  melancholy  instances  of  death  from  the  consequences  of  un- 
suspected retention,  while  the  early  and  frequent  use  of  the  catheter 
is  comparatively  innocuous.  It  is  especially  important  to  insist  on 
the  danger  that  the  practitioner  may  be  misled  by  the  patient,  who 
thinks  that  because  he  passes  urine  freely  he  necessarily  empties  his 
bladder  perfectly. 

Electricity  has  little  influence  on  the  chief  symptoms  of  tabes.  The 
voltaic  current  is  powerless  over  either  the  pains  or  the  ataxy,  whether 
it  be  applied  to  the  spine  or  the  limbs.  Faradisation  of  the  skin  by 
the  wire  brush  has  been  recommended  for  the  defect  of  cutaneous 
sensibility,  but  it  has  little  if  any  influence.  Temporary  improvement 
in  gait,  and  sometimes  a  little  permanent  improvement,  may  be 
obtained  by  faradisation  of  the  muscles ;  the  stimulation  of  the 
afferent  muscle-nerves  is  probably  the  mechanism  of  its  action,  but  I 
have  not  met  with  permanent  good  from  its  influence.  When  the 
bladder  or  its  sphincter  is  weak,  faradisation  from  the  hypogastrium 
to  the  perinseum  may  be  employed,  and  occasionally  seeijas  to  do  some 
good.  In  secondary  wasting  of  the  mviscles  an  attempt  may  be  made 
to  maintain  their  nutrition  by  stinlulating  them  with  whatever  current 
they  respond  to,  but  the  central  nature  of  the  cause  usually  renders 
local  treatment  ineffective.  Massage  also  has  been  recommended,  and 
possibly  may  be  beneficial  in  some  cases.  It  is  most  useful  if  com- 
bined with  the  systematic  use  of  the  exercises  in  co-ordination  devised 
by  Prenkel.* 

•  'Deutscli.  ined.  Wochenschr.,'  1896. 


PEIMAfiY    SPASTIC    PARAPLEGIA.  489 

Nerve- stretching  was  fashionable  a  few  years  ago,  the  operation 
being  usually  performed  on  the  sciatic  nerve.  In  the  first  case  in 
which  it  was  employed  (by  Langenbuch)  the  procedure  was  followed 
by  remarkable  and  mysterious  improvement,  not  only  in  the  pains, 
but  also  in  the  inco-ordination.  The  patient  subsequently  died  under 
chloroform,  administered  in  order  that  the  nei^es  of  the  anns  might 
be  stretched.  The  spinal  cord  was  found  by  Westphal  to  be  healthy. 
Although  the  nerves  were  not  examined,  there  can  be  little  doubt  that 
it  was  one  of  the  cases  in  which  the  nerves  alone  are  diseased.  The 
operation  has  since  been  performed  in  a  large  number  of  cases,  but 
without  results  that  have  secured  its  survival,  and  it  is  passing  into 
merited  disuse.  Its  place  was  taken  by  extension  of  the  spinal  column. 
In  this  procedure  the  patient  is  suspended  in  such  a  manner  as  to 
allow  the  weight  of  the  body  to  rest  on  the  head  and  shoulders,  and 
head  alone,  alternately,  and  the  ligaments  of  the  spinal  column  are 
supposed  to  be  so  stretched  that  the  spinal  cord  and  its  nerve-roots 
are  elongated.  The  chief  effect  is  produced  on  the  ligaments  of  the 
upper  part  of  the  spine,  but  the  actual  amount  of  elongation  of  the 
spine  is  exceedingly  slight  and  insignificant,  as  has  been  proved  by 
experiments  on  the  dead  body.  It  is  diflBcult  to  conceive  any  mecha- 
nism by  which  it  can  do  the  good  in  tabes  which  was  at  first  ascribed 
to  it ;  and  the  careful  observations  by  Eussell  and  Taylor  on  a  large 
number  of  cases  at  the  Queen  Square  Hospital*  seem  to  show  conclu- 
sively that  it  has  no  real  power  of  permanently  influencing  the  disease. 

It  is  important  that  the  patient  should  avoid  falls  as  far  as  possible, 
and  likewise  exposure  to  cold  and  wet.  Both  of  these  influences  occa- 
sionally excite  an  acute  exacerbation  of  the  pre-existiag  malady. 


PEIMARY   SPASTIC   PAEAPLEGIA 

(PEIMAET    LATERAL    SCLEEOSIS). 

The  morbid  state  thus  designated  is  one  that  has  been,  and  still  is, 
the  subject  of  much  discussion.  Its  relations  are  complex,  and  a 
somewhat  lengthy  pathological  introduction  is  necessary. 

General  Patholog-t. — We  have  seen  that  in  every  kind  of  trans- 
verse lesion  of  the  spinal  cord,  provided  this  is  situated  above  the 
lumbar  enlargement,  the  paralysis  of  the  legs  is  soon  accompanied  by 
excess  of  myotatic  irritability  (increased  knee-jerk,  foot-clonus),  and 
that  the  muscle-reflex  action  on  which  this  irritability  and  muscular 
tone  seem  to  depend,  gradually  increases  to  tonic  spasm,  so  that  a 
condition  develops  to  which  the  term  "  spastic  paraplegia  "  is  applied. 
A  condition  quite  similar  to  that  which  is  thus  secondary  to  a  trans- 
verse lesion  often  develops  gradually,  without  any  indication  of  a 
•  Russell  and  Taylor,  'Brain,'  Summer  Number,  J 890,  vol.  xiii,  p.  206. 


490  SPINAL    COED. 

i^rimary  focal  disease,  and  witliout  any  sensory  symptoms  to  indicate 
that  the  mischief  extends  beyond  the  purely  motor  elements  of  the 
cord.  We  have  also  seen  that  these  symptoms  indicate  disease  of  the 
upper  segment  of  the  motor  path,  the  cortico-spinal  segment  (p.  218), 
which  extends  from  the  motor  cortex  through  the  pyramidal  tracts, 
and  ends  in  the  grey  matter  of  the  cord,  doubtless  by  a  subdivision  and 
ramification  of  the  nerve-fibres  in  the  fibrillary  network  of  the  spongy 
substance.  Prom  the  gradual  onset  and  limitation  of  the  symptoms 
in  these  cases  it  has  been  assumed  that  the  disease  consists  of  a 
primary  sclerosis  of  the  pyramidal  tracts,  i.  e.  in  a  degeneration  of  the 
fibres  of  this  upper  segment.  Since  these  tracts  run  chiefly  in  the 
lateral  columns  the  disease  has  been  termed  "lateral  sclerosis." 
The  clinical  features  presented  by  these  cases,  and  their  probable 
significance,  were  first  pointed  out  by  Erb.*  Degeneration  of  the 
pyramidal  tracts  had  been  already  observed  by  Tiirck  (1856)  and 
Charcot  (1865).  Erb's  inference  as  to  the  nature  of  these  cases 
was  supported  by  the  independent  (and  indeed  previous)  researches 
of  Charcot  on  cases  of  muscular  atrophy ;  he  showed  that  in  such, 
cases  muscular  rigidity  coincides  with  degeneration  of  the  pyramidal 
tracts.  Pathologists  have  since  been  searching  for  confirmation  of  the 
hypotliesis — for  evidence  that  the  symptoms,  in  their  pure  form., 
without  muscular  atroi:)hy,  depend  on  degeneration  limited  to  the 
pyramidal  tracts.  Such  degeneration  in  slight  degree,  associated 
with  slight  symptoms,  has  been  found  in  cases  of  general  paralysis  of 
the  insane  (by  Westphal  and  others)  ;  but  in  all  other  cases  that  have 
been  examined  either  other  parts  of  the  white  substance  have  been 
degenerated,  or  the  disease  has  involved  also  the  anterior  cornua.f 
In  some  cases,  as  one  published  by  Dreschfeld  (of  which  a  figure  is 
given  at  page  497),  the  change  in  the  anterior  cornua  has  been  sa 
slight  that  the  required  conditions  are  nearly  fulfilled. J 

Although  absolute  demonstration  has  not  yet  been  furnished,  the 
indirect  evidence  of  the  correctness  of  the  pathological  hypothesis  is 
very  strong,  and  the  only  open  question  is  whether,  when  there  is  no 
disease  of  the  motor  nerve-cells  of  the  anterior  cornu,  the  degenera- 
tion is  so  limited  to  the  pyramidal  tracts  as  to  constitute  a  system 
disease  in  the  strict  sense  of  the  word.  It  is  not  surprising  that 
demonstration  of  the  nature  of  the  pure  cases  is  not  forthcoming, 
since,  as  we  shall  see,  the  disease  has  little  tendency  to  shorten  life. 
It  is  only  recently  that  it  has  been  shown  that  the  wasting  in  the 
anterior  horn  cells  which  underlies  progressive  muscular  atrophy  is 

*  In  1875,  'Berlin,  klin.  VVochenschr.,'  No.  26;  1877,  '  Virchow's  Arcliiv,' 
Bd.  Ixx. 

t  In  a  case  recorded  by  Stofella  no  other  part  than  the  pyramidal  tracts  conld 
be  seen  diseased  on  naked-eye  observation,  but  no  microscopical  examination  was 
made. 

J  The  same  is  true  of  several  cases  published  recently.  One  of  Striimpell's 
('  Ueut.  Zttichr.  f.  Nervenheilk.,'  1804,  v)  very  nearly  ful'iiled  the  requisite  condi- 
tions. 


PRIMARY   SPASTIC   PARAPLEGIA.  491 

not  necessarily  invariably  associated  witli  lateral  sclerosis,  and  there 
seems  no  reason  why  a  similar  degeneration  should  not  exclusively  affect 
the  upper  segments  of  the  motor  path  just  as  it,  rarely  no  doubt,  affects 
only  the  lower  segment.  In  the  majority  of  instances,  of  course,  both 
upper  and  lower  segments  are  affected  in  the  condition  known  as 
amyotrophic  lateral  sclerosis  (q.  v.). 

It  should,  moreover,  be  noted  that  cases  which  present  this  group 
of  symptoms  may  be  very  various  in  nature.  We  have  seen  (p.  254) 
that,  in  each  segment  of  the  motor  path,  the  same  symptoms  are 
produced  by  disease  of  any  part  of  the  segment.  In  the  upper 
segment,  with  which  we  are  now  concerned,  the  symptoms  are  the 
same,  wh'thei  the  disease  is  in  the  cortex  of  the  brain,  the  internal 
capsule,  the  pyramids  of  the  medulla,  or  the  pyramidal  tracts  of  the 
cord ;  and  they  must  also  be  the  same  if  the  disease  is  limited  to  the 
termination  of  the  segment  in  the  grey  matter  of  the  cord.  In 
cerebral  hemiplegia  the  state  of  the  arm  closely  resembles  that  present 
when  primary  spastic  paraplegia  involves  this  limb.  The  leg  in  hemi- 
plegia presents  a  less  close  resemblance  to  its  condition  in  spinal 
disease,  because  the  leg  is  innervated  from  both  cerebral  hemispheres^ 
and  the  supplementary  influence  of  the  hemisphere  of  the  same  side 
lessens  the  effect  of  the  disease  of  the  hemisphere  of  the  opposite  side. 
But  if  there  is  disease  of  the  leg-centres  in  both  hemispheres,  the 
state  of  the  legs  may  be  identical  with  that  resulting  from  disease  of 
the  spinal  cord.  Such  bilateral  disease  often  results  from  injury 
during  birth, — meningeal  haemorrhage  over  the  upper  part  of  the 
central  convolutions.  This  resulting  condition  is  termed  "  congenital 
spastic  paraplegia."     (See  Yol.  II.) 

On  the  other  hand,  it  is  extremely  probable  that  degeneration  begins 
in  the  termination  of  the  fibres  in  the  grey  matter,  just  as  it  does  in 
the  extremities  of  the  peripheral  nerves,  in  each  case  that  part  of  the 
neuron  furthest  from  its  nutritive  centre.  It  may  even  be  limited  to 
these  terminal  parts,  as  the  extremity  of  the  lower  segment  is  paralysed 
alone  by  many  agents,  typically  curara.  In  such  disease  the  white 
columns  would  be  found  normal,  as  in  one  recorded  case  in  which  the 
symptoms  of  spastic  paraplegia  existed  during  life,  and  no  anatomical 
change  was  discovered  after  death.  The  detection  of  the  disease  of  the 
terminal  portion  in  the  grey  matter  is  extremely  difficult,  perhaps  im- 
jjossible,  because  the  structure  must  consist  of  nerve-fibrillse  that 
interlace  with  others  that  are  unaffected.  This  view  is  supported  by 
the  fact  that  in  other  cases  some  degeneration  has  been  found  in  the 
lateral  pyramidal  tracts  in  the  lumbar  region  slighter  than  the  in- 
tensity of  the  symptoms  suggested,  and  gradually  lessening,  to  cease 
higher  up  the  cord.  Such  a  condition  is  comparable  to  the  degenera- 
tion of  the  lower  portion  of  the  second  segment  of  the  motor  tract  in 
some  cases  of  peripheral  (degenerative)  neuritis,  in  which  there  is  a 
degeneration  of  motor  nerve-fibres,  greatest  in  their  extremities,  ex- 
tending for  a  variable  distance  up  the  nerves,  but  lessening  long  before 


492  SPJNAL   COED. 

the  spine  is  readied.  It  is  Highly  probable  that  the  toxic  agents  which 
cause  lathjrism  and  pellagra  (q.  v.)  act  on  the  extremities  of  the 
pyramidal  fibres  as  curara  does  on  the  nerves;  among  the  spinal 
symptoms  of  lathyrism  are  paralysis  of  the  legs  with  rigidity  and 
contractures,  and  increase  of  myotatic  ii-ritability — foot-clonus,  &c.* 

Both  clinical  and  pathological  evidence  shows  that  the  morbid  state 
often  occui's  also  as  part  of  a  more  extensive  degeneration.  Degene- 
ration may  occur  in  both  lateral  and  posterior  columns,  giving  rise  to 
combined  paralysis  and  ataxy — "  ataxic  paraplegia,"  sufficiently  definite 
in  its  clinical  characters  and  course  to  make  its  distinction  convenient ; 
it  is  therefore  separately  described.  The  following  account  of  the 
clinical  features  of  spastic  paraplegia  is  founded  on  cases  in  which  the 
motor  paralysis  and  spasm  existed  alone,  with  no  defiaiite  sensory  loss, 
in  which  they  came  on  gradually,  with  nothing  in  the  state  of  the 
patient  or  the  history  of  his  symptoms  to  suggest  either  a  focal  lesion 
or  an  acute  process. 

Causes. — An  inherited  neuropathic  tendency  is  to  be  traced  occa- 
sionally in  this  as  in  other  chronic  spinal  diseases.  Thus  in  one  case 
there  was  a  history  of  insanity  in  uncle,  aunt,  and  two  cousins.  The 
disease  affects  both  sexes  in  almost  equal  frequency,  presenting  in  this 
a  contrast  to  posterior  sclerosis.  The  period  of  life  at  which  it  most 
frequently  begins  is  between  twenty  and  forty  ;  about  three  quarters 
of  the  cases  begin  in  these  two  decades,  and  about  an  equal  number 
in  each.  Cases  frequently  commence,  however,  in  the  second  decade 
of  life,  chiefly  in  its  latter  portion.  After  forty  they  become  much 
less  common ;  the  latest  age  at  which  I  have  known  a  typical  case  to 
commence  is  sixty-one. 

The  disease  sometimes  follows  syphilis  in  a  way  to  suggest  a  causal 
relation,  even  when  all  cases  are  excluded  in  which  there  is  any  reason 
to  suspect  a  focal  lesion.  In  one  case  the  symptoms  commenced  six 
months  after  the  primary  disease. f  But  this  antecedent  is  not  fre- 
quent, and  in  this  respect  also  the  disease  contrasts  with  posterior 
sclerosis.  Proximate  causes  are  to  be  traced  only  in  a  minority  of  the 
cases.  The  most  frequent  is  concussion  of  the  spine,  such  as  a  fall 
on  the  back.  Some  time,  often  two  or  three  years,  elapses  between 
the  fall  and  the  first  pronounced  symptoms  of  the  disease,  and  hence 
focal  lesions  due  to  the  fall  can  be  excluded.  Next  in  frequency 
is  repeated  exposure  to  ■  wet  cold.  Very  rarely  the  symptoms  have 
slowly  followed  some  acute  illness.  In  several  cases  the  disease  has 
succeeded  prostration  after  childbirth  or  abortion,  or  has  commenced 
during  lactation.  In  one  case  an  attack  of  arthritis  in  the  knee  and 
ankle  of  one  leg  was  the  immediate  antecedent,  and  this  leg  was  the 
first  to  become  weak.  In  another  case  the  symptoms  followed  sub- 
acute arthiitis  of  both  knee-joints,  apparently  rheumatic  in  nature. 

*  Sec  Marie,  '  Proj.  Med.,'  1883,  No.  43. 

t  The  cord  lesion  wms  found  to  be  sclerosis,  but  the  direct  cerebellar  tract 
was  also  degenerated  (Minkowski). 


PRIMARY    SPASTIC  PABAPLEOTa.  493 

The  possibility  that  a  joint  inflammatioii  may  be  of  spinal  origin  must 
be  borne  in  mind  in  considering  tbe  significance  of  sucb  cases,  but  it  is 
on  the  "whole  probable  that  a  primary  joint  affection  is  an  occasional 
cause  of  the  spinal  disease.*  (Acute  arthritis,  certainly  of  spinal 
oi'igin,  has  only  been  observed  in  severe  myelitis.)  The  cause  of  the 
congenital  foi-m  is  always  injury  to  the  brain  during  birth,  in  most 
cases  meningeal  haemorrhage,  causing  compression  of  the  motor 
cortex. 

Symptoms. — Weakness  of  the  legs,  of  very  gradual  development, 
is  the  first  symptom.  The  patient  finds  that  he  gets  tired  more 
readily  than  before,  and  that  the  legs  feel  heavy ;  sometimes  one  leg 
becomes  weak  before  the  other.  The  progress  of  the  weakness  is  very 
variable,  but  it  is  slow  in  all  characteristic  cases.  In  many  instances 
the  patient  is  still  able  to  walk  a  mile  or  two,  even  after  the  disease 
has  lasted  for  several  years,  slowly  increasing.  On  the  other  hand, 
walking  power  may  be  almost  lost  at  the  end  of  six  months.  It  is 
doubtful  whether  more  acute  cases  belong  to  this  category.  The  early 
weakness  is  often  accompanied  by  slight  unsteadiness,  chiefly  subjec- 
tive. When  the  patient  seeks  advice,  it  will  generally  be  found  that 
there  is  very  distinct  loss  of  power  in  the  flexors,  often  greatest  in  the 
flexors  of  the  hip,  but  considerable  also  in  those  of  the  knee  and  ankle, 
and  occasionally  much  greater  in  the  last  than  elsewhere.  The 
knee-jerk  is  excessive  and  quick ;  the  rectus  contraction  can  be 
obtained,  as  the  patient  lies,  by  tapping  the  depressed  patella,  and  a 
rectus-clonus  is  often  obtainable  by  sudden  depression  of  the  patella. 
The  foot-clonus  is  usually  also  obtained  with  readiness.  In  rare 
cases,  in  which  the  upper  part  of  the  legs  suffers  most,  there  may  be 
a  rectus-clonus,  but  only  slight  indications  of  a  foot-clonus,  two  or 
three  movements  quickly  ceasing.  The  tendency  to  spasm  is  at  first 
noticeable  as  slight  stiffness  of  the  legs  on  first  rising  in  the  morning, 
but  it  gradually  increases  in  degree  as  power  lessens,  until  at  last  the 
legs,  whenever  extended,  pass  into  a  condition  of  strong  extensor 
spasm,  r^dly  fixing  them  to  the  pelvis,  so  that,  as  the  patient  lies,  if 
one  leg  is  hfted  from  the  couch  by  the  observer,  the  other  leg  is 
moved  also.  The  spasm  may  be  such  that  the  knee  cannot  be  pas- 
sively flexed  by  any  force  that  can  be  applied  to  it  until  the  spasm  has 
become  less.  When  flexed  the  limb  is  comparatively  supple ;  but 
if  it  is  then  extended,  the  spasm  instantly  returns,  making  the  limb 
rigid,  and  often  completing  the  extension,  just  as  the  blade  of  a  knife 
opens  out  under  the  influence  of  its  spring,  "clasp-knife  rigidity." 
The  spasm  is,  roughly  speaking,  proportioned  to  the  loss  of  power,  and 
its  extensor  character  may  enable  the  patient  to  stand,  the  legs  being 
fixed  and  rigid,  when  muscular  power  is  quite  insufficient  to  support 
the  body  when  it  is  unaided  by  the  spasm.  In  a  still  greater  degree 
a  voluntary  effort  may  only  excite  general  spasm  of  the  limb  instead  cf 
causing  k  defijiite  movement.     Occasionally  there  are  also  paroxysms 

*  See  Arthritic  Muscular  Atrophy. 


494  SPINAL   CORD. 

of  brief  flexor  spasm  causing  tlie  legs  to  draw  up.  This  occurs  cMefly 
wlieii  the  patient  is  in  bed,  and  especially  during  sleep.  When  the 
spasm  is  great,  a  paroxysm  may  pass  into  violent  clonic  spasm,  but 
this  is  merely  the  same  clonus  that  can  be  produced  by  passive  exten- 
sion, excited  by  the  tension  of  the  spontaneous  spasm  (see  p.  263). 
Reflex  action  from  the  skin  is  also  usually  excessive,  and  the  stimulus 
excites  an  attack  of  spasm. 

The  gait  of  the  sufferers  from  spastic  paraplegia  is  very  character- 
istic. The  legs  seem  to  drag  behind  the  patient,  and,  in  walking, 
each  is  hauled  forwards  as  a  rigid  whole,  the  toes  catching  against  the 
ground,  and,  when  the  ball  of  the  foot  rests  on  the  ground,  the  limb 
may  shake  from  the  clonus  developed  by  the  extension  of  the  calf 
muscles.  When  the  patient  sits,  a  similar  trepidation  occurs  until 
the  patient  pushes  the  leg  forwards  so  that  the  heel  rests  also  on  the 
floor.  The  muscles  of  the  legs  are  usually  large  and  well  nourished. 
They  often  seem,  indeed,  to  be  hypertrophied,  and  perhaps  are  reaUy 
so;  the  contraction  of  spasm  constitutes  a  physiological  stimulus  to 
growth,  just  as  does  voluntary  effort.  But  they  are  not  always  large ; 
they  are  sometimes  moderately  wasted.  The  electric  irritability  is 
usually  perfectly  normal,  but  in  the  muscles  that  present  the  slight 
wasting  just  mentioned  there  may  be  a  trifling  diminution  of  irrita- 
bility to  f aradism  and  voltaism  alike.  When  the  patient  ceases,  or 
almost  ceases  to  walk,  contracture  sometimes  occurs  in  the  calf 
muscles.  An  active  contracture  is  indeed  common,  preventing  at  first 
passive  flexion  of  the  ankle  beyond  a  right  angle ;  but  if  the  pressure 
is  maintained,  the  muscles  yield  and  full  flexion  is  possible.  But  in 
some  cases  there  is  the  fixed  contracture  described  on  p.  257  (form  3). 
Flexor  contraction  at  the  hip  or  knee  may  occur  also  in  the  same  cases 
under  the  influence  of  posture,  but  is  on  the  whole  rare,  the  tendency 
to  it  being  counteracted  by  the  extensor  spasm. 

The  arms  are  often  unaffected,  but  they  suffer  in  some  cases,  and 
present  the  same  progressive  weakness  and  excess  of  myotatic  irrita- 
bility. Vigorous  contractions  are  elicited  by  a  tap  on  the  tendon  of  a 
muscle,  or  on  the  bone  to  which  the  tendon  is  attached,  provided  the 
muscle  is  extended.  A  clonus  can  sometimes  be  obtained  in  the  flexors 
of  the  fingers.  There  is  less  paroxysmal  spasm  than  in  the  leg,  but 
much  rigidity  of  the  limb,  from  active  muscular  contracture,  flexing  the 
elbow  and  flexing  the  fingers  at  all  the  joints,  as  in  the  "  late  rigidity" 
of  hemiplegia.  The  difference  in  the  form  of  spasm  in  the  arm  and  leg 
is  no  doubt  connected  with  existence  of  a  reflex  extensor  mechanism, 
in  the  lumbar  centres,  concerned  in  the  act  of  standing.  The  muscles 
of  the  arm  are  often  as  well  nourished  as  those  of  the  leg.  The  affec- 
tion of  the  two  arms  is  very  rarely  equal ;  it  is  common  for  one  arm 
to  be  almost  normal  when  the  other  is  much  affected  and  both  legs 
are  equally  involved.  Sometimes,  however,  the  arm  and  leg  on  one 
side  are  paralysed,  and  the  limbs  on  the  other  side  are  so  slightly 
involved  that,  in  comparison  with  the  jpalsied  members,  they  seem 


PRIMARY    SPASTIC  PARAPLEGIA.  495 

normal  ,•  the  patient  often  belieyes  that  they  are  unaffected,  but 
examination  always  reveals  some  weakness .  and  an  abnormal  degree 
of  myotatic  irritability. 

The  muscles  of  the  trunk  may  also  suffer.  A  subjective  "  feeling  of 
weakness  "  in  the  back  is  often  complained  of  early,  but  seems  to  be 
as  much  a  sensory  as  a  motor  symptom.  Painful  spasm  of  the 
abdominal  muscles  may  occur  in  severe  cases,  and  I  have  even  known 
attacks  of  tetanoid  rigidity  of  the  back  to  be  produced  by  attempts  to 
move. 

It  is  not  common,  in  typical  cases,  for  the  symptoms  to  extend  into 
the  region  of  the  cranial  nerves,  but,  in  rare  instances,  dif&culty  of 
swallowing  and  of  articulation  has  existed,  due  to  a  similar  palsy  of 
the  bxJbar  nerves.  Excessive  myotatic  iiTitability  has  also  been 
observed  in  the  muscles  of  mastication,  so  that  a  tap  on  the  chin 
causes  a  vigorous  elevation  of  the  depressed  jaw  (Beevor). 

Sensory  symptoms  are  often  entirely  absent,  with  the  exception  of 
slight  dull  pains  in  the  legs,  or  more  frequently  in  the  back.  The 
sensation  of  weakness  in  the  back,  already  mentioned,  occasionally 
reaches  an  intense  degree.  Eheumatoid  pains  may  be  troublesome, 
but  sharp  pain  is  rare,  except  in  untypical  cases  to  be  presently 
mentioned.  In  such  cases  also  there  may  be  very  slight  defect  of 
sensibility — such,  for  instance,  as  blunting  of  tactile  sensibility  on 
the  finger-tips.  Subjective  sensations  of  "numbness,"  tingling, 
formication,  are  more  common.  They  may  exist  for  years  without 
the  development  of  any  other  anaesthesia,  in  spite  of  the  progress  of 
the  motor  symptoms. 

The  s]3hincters  are  sometimes  affected,  occasionally  very  early; 
often,  however,  they  escape  even  to  an  advanced  stage.  Sexual  power 
may  be  lost,  or  may  remain,  even  when  the  spastic  paraplegia  has 
reached  a  high  degree.  The  nutrition  of  the  skin  and  joints  under- 
goes no  change. 

Ocular  symptoms  are  rare,  with  the  exception  of  nystagmus,  which 
will  often  be  found  if  searched  for.  The  action  of  the  pupils  is 
usually  perfect,  and  I  have  only  once  seen  optic  nerve  atrophy  in  an 
uncomplicated  case. 

The  infantile  form  may  resemble  very  closely  that  which  occurs  in 
adults.  There  are  the  same  extensor  spasm  and  increase  of  all  forms 
of  reflex  action.  As  the  child  sits  on  the  knee  or  a  chair  any  sensory 
stimulus  will  make  the  legs  shoot  out  in  spasm  (Fig.  131).  But  the 
rigidity  does  not  reach  the  extreme  degree  often  attained  in  the  common 
form.  The  excess  of  the  knee-jerk  is  always  distinct,  but  a  foot- 
clonus  is  not  often  to  be  obtained,  perhaps  because  the  muscle-reflex 
mechanism  related  to  the  calf  muscles  has  not  received  the  functional 
development  that  must  result  from  the  process  of  walking,  with  its 
recurring  sequence  of  tension  and  contraction.  The  active  contracture 
in  the  calf  muscles,  which  most  cases  present,  is  a  serious  hindrance 
to  walking  even  when  the  muscular  power  is  sufficient,  and  it  is  long 


496 


SPINAL   COED. 


before  the  attempt  overcomes  the  contracture.  In  most  cases,  how- 
ever, the  child  ultimately  gains  the  power  of  walking,  although  much 
later  than  normal,  and  often  with  some  peculiarity  of  gait,  sometimes 
a  tendency  to  "  cross-legged  progression,"  in  which  one  foot  gets  over 
or  in  front  of  the  other  (Fig.  132),  or  with  a  swinging  oscillation  of 

Fig.  132. 


Wia.  131. 


Fig.  131. — Congenital  spastic  paralysis  (cerebral).  Extensor  spasm  in 
the  legs  excited  by  a  sensory  impression.  (Drawn  by  Dr.  H.  R.  Spencer, 
from  a  photograph  by  Mr.  Hyde  Marriott.) 

Fig.  132. — Infantile  spastic  paralysis  of  cerebral  oricrin :  cross-legged 
progression.     (Drawn  by  Dr.  Spencer,  from  a  photograph.) 

the  body,  which  may  persist  to  adult  life.  The  growth  of  the  legs  is 
often  hindered. 

The  arms  do  not  present  tonic  spasm  such  as  is  seen  in  adult  cases. 
There  may  be  a  choreoid  disorder  of  movement,  spontaneous  irregular 
movements  with  inco-ordination,  but  in  the  cases  that  can  fairly  be 
called  "  spastic  paraplegia "  the  arm  symptoms  are  slight.  When 
considerable  the  condition  is  often  termed  "double  athetosis."  Its 
characters  are  described  in  Vol.  II. 

Transitional  forms  are  met  with  which  constitute  gradations  between 
primary  spastic  paraplegia  and  other  degenerations  of  the  spinal  cord. 
Slight  inco-ordination  may  co-exist  in  cases  approximating  the  "  ataxic 
paraplegia  "  described  in  the  next  section.  Cases  may  begin  as  pure 
tabes,  and  indications  of  lateral  sclerosis  may  be  superadded.  Slight 
muscular  wasting  in  the  arms  may  be  associated  with  indications  of 
lateral  sclerosis  in  the  legs,  and  constitute  a  transition  to  the  form  of 
spinal  muscular  atrophy  to  which  the  name  "amyotrophic  lateral 
sclerosis "  has  been  given.  Yery  rarely  considerable  muscular 
wasting  succeeds  spasm  in  the  same  part.  Spastic  paraplegia  also 
is  often  the  early  stage  in  cases  which  afterwards  develop  other 
symptoms  characteristic  of  disseminated  sclerosis.      Nystagmus,  how* 


PRIMARY    SPASTIC   PARAPLEGIA. 


497 


ever,  as  lias  already  been  stated,  may  occur  in  cases  otherwise  offering 
no  symptoms  except  those  of  lateral  column  affection.  Unequivocal 
symptoms  of  syringomyelia  have  also  been  known  to  develop  in  a* 
case  which  presented  in  its  earlier  stages  the  characteristic  signs  of 
primary  lateral  sclerosis.* 

Lateral  sclerosis  of  the  spinal 
cord  (like  posterior  sclerosis) 
often  forms  part  of  the  morbid 
process  that  underlies  general 
paralysis  of  the  insane.  In  some 
cases  of  this  character  the  mental 
symptoms  are  extremely  slight, 
and  the  case  may  have  the 
aspect  of  a  primary  spinal  dis- 
ease, with  slight  mental  failure 
complicating  it. 

The  course  of  characteristic 
cases  of  primary  spastic  para- 
plegia is  thus  very  chronic.  The 
malady  may,  at  any  stage,  cease 
to  advance.  Slight  symptoms 
may  remain  stationary  for  twenty 
years.  Often,  however,  arrest 
only  occurs  when  the  disease 
has  reached  a  considerable  de- 
gree. It  is  perhaps  the  least 
dangerous  to  life  of  any  chronic 
spiaal  disease.  Even  secondary 
kidney  trouble  scarcely  ever  oc- 
curs; perhaps  the  excessive  re- 
flex action  may  save  the  bladder 
from  injurious  over-distension. 
It  is  when  other  elements  of 
the  cord  suffer  that  dangerous 
complications  ensue. 

PATHOLOGICAIi  AkATOMT. — In 

the  fact  just  stated  we  probably 
have  an  explanation  of  the  silence  of  morbid  anatomy  on  the  subject  of 
uncomplicated  lateral  sclerosis.  "  Nee  silet  mors,"  the  apt  motto  of  the 
Pathological  Society,  is  true  chiefly  of  disease  that  kills.  Complete 
degeneration  of  the  pyramidal  tracts,  anterior  as  well  as  lateral,  is 
met  with  chiefly  in  cases  in  which  the  anterior  ganglion-cells  and  motor 
nerves  are  also  diseased,  although  in  some  instances  the  amount  of 
this  disease  is  small,  and  limited  to  the  cervical  region.    Aa  instance 

*  Charcot,  '  Progrfts  M6dicaV  1891. 

t  I  am  indebted  for  these  sections  to  Dr.  Dreschfeld,  who  has  published  the  case 
(«  British  Med.  Journal.,*  Jan.  29fch,  1881). 

VOL.  1.  32 


Pia.  133. — Sclerosis  of  the  lateral  and  (in 
the  cervical  region  anterior)  pyramidal 
tracts,  with  slight  degeneration  of  the 
anterior  cornua.  A,  cervical;  B,  dor- 
sal; C,  lumbar  sections.f 


498 


SPINAL    CORD. 


of  sucli  degeneration  is  shown  in  Fig  133,  Tlie  degeneration  in  the 
lumbar  enlargement  is  usually  limited  to  the  lateral  tract,  but  the 
anterior  tract  may  be  affected  higher  up  the  cord,  as  in  Fig.  133,  A.  The 
lateral  sclerosis  is  bounded  externally,  in  the  dorsal  and  cervical 
regions,  by  the  normal  direct  cerebellar  tract,  and  internally  by  the 
narrow  "  lateral  limiting  layer  "  which  intervenes  between  the  pyra- 
midal tract  and  the  intermediate  grey  matter  There  is  the  usual 
increase  of  connective  tissue  and  wasting  of  nerve-fibres.  In  many 
cases  granule-cells  are  abundant  in  the  affected  area.  They  are 
always  present  in  cases  of  short  duration,  and  indicate  the  stage 
rather  than  the  nature  of  the  process.  The  degeneration  probably 
begins  in  all  cases  in  the  nerve-elements  themselves.  In  most  cases 
many  nerve-fibres  can  still  be  seen,  scattered  through  the  sclerosed 
area;  most  of  them  are  fibres  which  lie  m  the  pyramidal  tract, 
but  do  not  belong  to  it.  The  degeneration  of  the  tract  has  been 
traced  through  the  medulla,  pons,  and  cerebral  hemispheres  to  the 
motor  cortex,  in  which  indications  of  degeneration  have  also  been 
found.  This  degeneration  through  the  brain  was  traced  in  one  case 
in  which  the  disease  of  the  anterior  cornua  was  slight,  the  wasting 
was  limited  to  the  hand  muscles,  and  the  spastic  paralysis  began  in 
the  legs  and  then  invaded  the  arms.*  Such  a  case  is  almost  a  pure 
degeneration  of  the  whole  of  the  first  segment  of  the  motor  path. 


FlO.  134. — Sclerosis  of  the  pyramidal  tracts,  lateral  and  anterior:  dorsal 
region.     From  a  cd,se  of  muscular  atrophy. 

The  degeneration  of  the  pyramidal  tracts  is  well  shown  in  Fig.  134, 
from  a  case  of  progressive  muscular  atrophy.  The  anterior  and 
lateral  tracts  are  entirely  degenerated,  and  the  sclerosis  stops  ab- 
ruptly at  the  outer  margin  of  the  lateral,  but  in  front  extends  for« 

•  Kojewnikoflf,  'Arch,  de  Neurologie,'  1883,  No.  18. 


PRIMARY    SPASTIC   PARAPLEGIA.  499 

wards,  probably  in  the  short  fibres  that  connect  the  anterior  comu 
at  different  levels.  In  other  cases,  in  which  the  anterior  ganglion- 
cells  were  normal,  the  sclerosis  has  not  been  limited  to  the  pyra- 
midal tracts ;  the  anterior  columns  have  been  also  sclerosed  in  the 
lumbar  region  of  the  cord.*  The  direct  cerebellar  tract  and  pos- 
terior vesicular  columns  have  been  also  found  degenerated  with  the 
pyramidal  tract,  and  an  annular  sclerosis  of  the  periphery  of  the 
antero-lateral  column  has  been  found  in  several  cases. f  More  frequent 
still  is  a  combination  with  degeneration  of  the  posterior  columns, 
described  more  fully  in  the  next  section.  In  most  of  these  combined 
cases  the  degeneration  has  lessened  in  the  upper  part  of  the  cord.  In 
one  case,  at  least,  the  white  columns  of  the  cord  have  been  found 
healthy.  The  probable  significance  of  this  fact  has  been  already  stated 
(p.  491).  It  should  be  remarked,  however,  that  there  seems  sometimes 
to  be  a  tendency  for  the  morbid  process  to  extend  beyond  the  pyra- 
midal tracts  in  a  manner  that  suggests  a  greater  tendency  for  the 
secondary  connective-tissue  changes  to  take  on  an  independent 
invasive  activity  than  in  other  system  diseases.  (See  also  Ataxic 
Paraplegia.)  Another  point  that  deserves  mention  is  the  occasional 
occurrence  of  lateral  sclerosis  in  association  with  insular  sclerosis.  In 
some  cases  of  this  character  the  degeneration  of  the  pyramidal  tracts 
is  purely  secondary,  the  result  of  the  damage  to  the  pyramidal  fibres 
by  an  islet  of  sclerosis  situated  in  some  part  of  their  course.  But  it 
does  not  appear  that  this  explanation  can  be  given  of  all  cases.  In 
Bome  instances  the  sclerosis  of  the  pyramidal  tract  appears  to  be  inde- 
pendent and  coincident.  A  similar  association  of  insular  and  posterior 
sclerosis  has  also  been  observed ;  and  such  combinations  receive 
additional  emphasis  from  the  fact  that  insular  sclerosis  may  be 
accompanied  by  a  primary  atrophy  of  the  optic  nerve,  similar  to  what 
occurs  in  tabes,  although  probably  not  identical.  These  facts  make  it 
probable  that  lateral  sclerosis,  although  a  system  disease,  is  often  one 
of  less  strict  features  than  tabes,  having  some  tendency  towards 
chronic  myelitis.  We  shall  see  that  this  is  true  also  of  ataxic 
paraplegia. 

The  probable  mechanism  of  the  symptoms  that  give  the  dominant 
characters  to  the  malady  have  been  considered  in  the  general  account 
of  the  symptoms  of  disease  of  the  spinal  cord.  The  most  important  fact 
is  that  the  degeneration,  whatever  its  upward  extent,  always  involves 
the  lowest  part  of  the  pyramidal  segment,  because  it  seems  to  begin 
ill  this  and  ascend  the  fibres.  Hence  the  intra-cornual  termination 
must  always  be  involved.     This,  it  is  assumed,  is  the  structure  that 

*  As  in  a  case  recorded  as  one  of  spastic  paraplegia  by  Hopkins  ('Brain,'  Oct., 
1883),  but  this  was  an  example  of  combined  lateral  and  posterior  sclerosis  (ataxic 
parMplegia).  An  illustration  of  the  changes  in  this  case  is  given  in  the  next 
section. 

t  Direct  cerebellar  and  pyramidal  tracts  in  a  case  by  Minkowski,  in  which  the 
disease  rapidly  succeeded  syphilis j  annular  scljrosis  by  Westphal  (see  Ataxic 
Paraplegia). 


500  SriNAL   COED. 

controls  the  muscle-reflex  centre,  and  the  consequent  loss  of  control 
explains  the  excess  of  myotatic  irritability  and  the  progressive  spasm, 
progressive  by  what  may  be  termed  a  functional  hypertrophy  result- 
ing from  continuous  over-action.  When  there  is  the  rare  consecutive 
wasting  of  muscles  we  must  conceive  that  the  degeneration  involves  also 
the  ganghon-cells  and  lower  segment  of  the  motor  path  ;  when  there 
is  coincident  wasting  of  other  muscles  than  those  that  aro  the  seat  of 
spasm,  it  would  seem  that  some  elements  of  this  lower  segment  are 
the  seat  of  a  primary  degeneration.  The  slighter  muscular  wasting 
without,  or  with  only  trifling,  change  in  electrical  irritability,  is  the 
expression  of  a  slighter  alteration  in  the  nutrition  of  the  cells  and  fibres, 
without  actual  destructive  degeneration. 

Diagnosis. — The  diagnosis  rests  on  the  combination  of  weakness, 
excess  of  myotatic  irritability,  and  spasm — on  the  gradual  onset  of 
these  symptoms — and  also  on  the  absence  of  indications  of  a  focal 
lesion.  An  acute  onset,  occupying  a  few  days  or  weeks,  is  prima 
facie  evidence  of  a  lesion  that  takes  the  case  out  of  the  category  of 
degenerative  disease.  In  most  acute  cases  there  is  other  evidence  of  a 
focal  lesion,  extending,  at  some  level,  beyond  the  limits  of  the  motor 
path.  Such  indications  are  initial  impairment  of  sensation,  or  a 
girdle-pain.  The  latter  indicates  irritation  of  the  posterior  root-fibres 
at  a  certain  level,  and  proves  that  at  that  level  the  disease  extends 
beyond  the  limits  of  the  pyramidal  tracts.  Spastic  paraplegia  is 
common  after  such  lesions,  but  is  secondary  and  not  primary. 

Although  marked  sensory  symptoms,  in  a  case  that  presents  the 
symptoms  and  course  of  primary  lateral  sclerosis,  are  thus  evidence 
that  the  sclerosis  extends  beyond  the  motor  tracts,  and  that  the  case 
is  not  one  of  pure  lateral  sclerosis,  it  is  doubtful  what  significance 
is  to  be  attached  to  very  slight  sensory  symptoms,  such  as  slight  sub- 
jective sensations  of  dull  pain,  formication,  &c.,  when  they  exist  alone. 
It  is  possible  that  they  are  due  to  functional  disturbance  in  the  sen- 
sory nerve-elements,  and  do  not  imply  structural  disease  outside  the 
motor  area.  They  are  not  followed  by  any  more  pronounced  sensory 
symptoms. 

The  absence  of  objective  unsteadiness  is  the  chief  distinction  from 
ataxic  paraplegia  (q.  v.).  When  this  is  slight  it  may  be  difficult  to  say 
in  which  class  a  case  should  be  placed.  There  are  no  doubt  intermediate 
cases  in  which  there  is  a  very  slight  degree  of  the  additional  lesion  that 
exists  in  ataxic  paraplegia,  but  the  distinct  objective  character  of  the 
ataxy  is  the  best  criterion.  Considerable  muscular  wasting  in  any  part 
is  commonly  regarded  as  bringing  the  case  into  another  category,  that 
of  "  amyotrophic  lateral  sclerosis  " ;  but  some  cases  of  the  kind,  in 
which  the  wasting  is  confined  to  a  few  muscles,  resemble  more  closely 
the  cases  we  are  now  considering  than  they  do  those  with  wide-spread 
and  extreme  muscular  atrophy.  These  cases  are  further  considered 
in  the  account  of  progressive  muscular  atrophy. 

Primary  spastic  paralysis  is  not  uncommon  in  those  who  are  at  the 


PRIMARY    SPASTIO    PARAPLEGIA.  501 

age  and  of  tlie  sex  at  which  hysteria  prevails,  and  there  is  no  form  of 
cord  disease  that  is  so  often  mistaken  for  hysterical  paraplegia.  The 
mistake  is  facilitated  by  the  perfect  muscular  nutrition.  But  the 
mistake  ought  not  to  occur,  as  the  peculiar  extensor  character  of  the 
spasm,  lessening  with  flexion,  is  distinctive ;  nothing  resembling  it 
ever  occurs  in  hysterical  paraplegia.  When  the  spasm  is  trifling  or 
absent,  as  in  slight  and  early  cases,  the  diagnostic  difficulty  is  much 
greater,  and  is  increased  by  the  fact  that  slight  excess  of  myotatic 
ii-ritability  occurs  in  some  cases  of  so-called  hysterical  paralysis.  But 
this  scarcely  ever  reaches  the  degree  necessary  to  give  rise  to  a  true 
foot-clonus  or  a  rectus-clouus.  There  may  be  a  slight  clonus  pro- 
duced through  a  voluntary  depression  of  the  foot  of  the  patient  in 
response  to  the  passive  flexion  of  the  ankle,  and  readily  recognised ; 
or  a  true  clonus  may  be  obtainable  during  hysterical  contracture,  but 
apart  from  such  contracture  a  true  foot-clonus  or  a  rectus-clonus 
•deserves  the  greatest  weight,  as  all  but  conclusive  evidence  of  organic 
disease.  I  have  known  many  mistakes  in  diagnosis,  in  which  lateral 
sclerosis  was  mistaken  for  hysterical  paraplegia  owing  to  disregard  of 
the  evidence  afforded  by  this  symptom,  but  I  have  never  known  the 
opposite  error  from  xmdue  regard  to  this  symptom.  Moreover 
an  excess  of  myotatic  irritability  in  so-called  hysterical  paralysis  must 
depend  on  more  than  functional  disease.  There  must  be  changes  in 
nutrition,  and  consequent  persistent  defective  control  of  the  muscle- 
reflex  centres.  On  the  hypothesis  that  I  have  advanced,  this  control 
is  exerted  by  the  termination  of  the  pyramidal  fibres,  i.  e.  of  the  upper 
motor  segment,  by  the  structures  degeneration  of  which  probably 
■causes  spastic  paraplegia.  A  case  is  actually  on  record  (the  case  of 
lateral  sclerosis  described  by  Charcot  in  1865)  in  which  an  initial 
hysterical  paraplegia,  cured  suddenly  and  relapsing  on  emotion, 
passed  ultimately  into  lateral  sclerosis.  Similar  cases  of  initial 
hysterical  jDaraplegia  frequently  develop  all  the  characters  of  dis- 
seminated sclerosis. 

When  the  arm  and  leg  suffer  on  one  side  only,  the  disease  may  be 
mistaken  for  cerebral  hemiplegia.  There  is  not,  however,  any  affection 
of  the  face,  which,  although  theoretically  conceivable,  is,  as  a  matter 
of  fact,  always  absent.  The  limbs  on  the  other  side  are  never  quite 
normal,  but  present  slight  symptoms — ^weakness  and  an  excess  of 
myotatic  irritability — similar  to  those  on  the  affected  side,  which 
indicate  the  nature  of  the  malady. 

The  diagnosis  of  the  congenital  infantile  form  is  only  difficult  when 
the  observer  is  unaware  of  the  occurrence  of  these  cases.  A  slight 
degree  of  inco-ordination  in  the  hands  will  usually  be  found  if  they  are 
carefuUy  watched  while  the  patient  takes  hold  of  some  object.  The 
wide  separation  and  irregular  movement  of  the  fingers  is  very  charac- 
teristic. Chronic  primary  cord  diseases  are  almost  unknown  in  young 
children.  Caries  of  the  spine  is,  in  them,  the  chief  cause  of  para- 
plegia ;  and  the  definite  onset  of  the  paralysis,  in  a  previously  healthy 


502  SPINAL   COED. 

child,  is  an  absolute  distinction  from  tlie  cases  of  birth-palsy.  Other 
diagnostic  indications  are  described  in  Vol.  II  (Infantile  Meningeal 
Hsemorrhage) .  A  definite  onset  also  distinguishes  ghoma  of  the 
pons,  which  may  likewise  cause  spastic  paralysis  of  arms  and  legs. 

Pbognosis. — In  primary  lateral  sclerosis  there  is  some  prospect  of 
arrest,  and  even  of  improvement,  if  the  disease  has  not  reached  an 
advanced  stage.  Actual  recovery  is  rare,  but  does  sometimes  occur  in 
early  cases.  When  the  spastic  state  is  well  developed  and  has  lasted 
for  some  time  it  is  verj  rare  to  obtain  more  than  arrest.  This  is, 
peihaps,  due  rather  to  the  tendency  of  the  disease  than  to  its  degree, 
since  a  similar  condition  that  is  secondary  to  a  focal  lesion  often 
passes  away  completely;  and  many  facts  show  that  it  is  not  likely 
that  there  is  anything  in  the  nature  of  the  lesion  to  preclude  the 
restoration  of  a  normal  condition  until  dense  connective  tissue  has 
replaced  the  nerve-elements.  In  the  cases  that  improve  most,  it  is 
possible  that  the  disease  is  limited  to  the  terminal  structures.  We 
have  not,  at  present,  any  guide  to  the  prognosis  in  an  individual  case, 
besides  its  duration  and  degree,  except  its  observed  tendency,  especially 
under  treatment.  The  prognosis  of  the  infantile  form  is  considered 
in  Vol.  II. 

Treatment. — So  far  as  drugs  are  concerned,  the  treatment  is,  to  a 
large  extent,  the  same  as  that  of  posterior  sclerosis,  already  described. 
The  drugs  most  useful  are  the  same,  but,  unfortunately,  their  influence 
is  less  frequently  appreciable.  Nux  vomica  and  strychnine  have,  how- 
ever, to  be  given  with  caution,  and  in  very  minute  doses,  as  they  have 
a  tendency  to  increase  the  spasm.  In  severe  cases  this  is  a  most 
distressing  symptom,  and  often  not  amenable  to  any  influence. 
Bromide  sometimes  lessens  it  slightly,  but  even  large  doses  of 
bromide  have  but  a  trifling  effect.  Indian  hemp,  belladonna,  and 
Calabar  bean  may  be  tried,  but  seldom  have  a  distract  influence.  One 
of  them  may  be  combined  with  arsenic,  or  whatever  metallic  agent  is 
employed.  Absolute  rest  is  sometimes  of  service,  and  occasionally 
seems  to  produce  improvement,  which  all  treatment  failed  to  eifect 
while  the  patient  was  walking  about.  The  avoidance  of  fatiguing^ 
exertion  is  a  very  important  element  in  treatment,  and  often  seems  ta 
permit  medicines  to  do  good,  which  before  were  counteracted.  Rub- 
bing is  also  beneficial  in  some  cases.  Its  influence  on  the  spasm  i& 
often  very  distinct  during  the  process,  and  a  long  course  of  rubbing 
has  produced  a  permanent  improvement  in  the  spastic  condition. 
Upward  rubbing  seems  to  have  more  influence  than  kneading  the 
muscles.  If  there  is  contracture  of  the  calf -muscles,  the  foot  should 
be  pressed  up  while  these  muscles  are  rubbed.  When  there  are 
facilities  for  it,  the  rubbing  may  advantageously  be  combined  with 
sweating  in  the  Turkish  bath.  In  one  case,  of  moderate  degree,  almost 
all  the  symptoms  passed  away  after  a  long  course  of  Turkish  baths, 
arsenic  being  also  given.  The  patient,  who  could  at  first  walk  scarcely 
half  a  mile,  became  able  to  walk  several  miles  without  fatigue,  and  the 


ATAXIC   PARAPLEGIA.  503 

improyement  was  permanent.  Electricity  is  useless  in  the  pure  disease, 
Fai'adism  and  all  painful  applications  are  harmful,  stimulating  further 
the  already  excessive  excitability  of  the  reflex  centres.  The  constant 
current  to  the  muscles,  or  from  the  spine  to  the  muscles  or  to  the  feet 
in  water,  has  no  distinct  influence  in  either  lessening  the  spasm  or 
improving  the  strength.  The  actual  cautery  is  in  many  cases  most 
beneficial. 

In  the  infantile  form,  drugs  are  useless.  "Rubbing  is  desirable,  and 
can  be  efficiently  performed  by  the  nurse  or  mother.  Carefidly  planned 
gymnastic  exercises  are  also  useful.  The  tendo  Achillis  is  sometimes 
divided  for  the  contracture  of  the  calf  muscles,  but  the  operation  is 
useless  and  ought  never  to  be  performed.  Supports  help  the  child  to 
walk  somewhat  sooner  than  it  would  without  their  aid,  and  so  hasten 
improvement,  but  they  should  only  be  employed  when  there  is  sufficient 
power  and  control  to  make  them  useful  by  enabling  the  will  to  effect 
ordered  movements. 

Family  Form:  op  Spastic  Paralysis. 

Cases  in  which  the  usaal  symptoms  of  spastic  paralysis  have  been 
present  in  several  members  of  the  same  family,  occasionally  in  different 
generations,  have  been  described  by  Erb,*  Striimpell.t  Bernhardt,! 
!N"ewmark,§  Raymond,  Sougues,||  G-ee,^  Tooth,**  and  others.  Occa- 
sionally to  the  symptoms  of  the  cord  affection  evidence  of  bulbar 
involvement  has  been  added,  and  the  condition  has  probably  been 
dependent  upon  some  congenital  weakness  of  the  pyramidal  tracts 
and  their  correlated  structures,  predisposing  them  to  premature  decay. 
Optic  atrophy  has  also  been  met  with.  The  condition  is  f  ui'ther  briefly 
alluded  to  in  the  chapter  on  Hereditary  Ataxy  (j.  v.). 


ATAXIC   PAEAPLEaiA. 

(combined   LATEEAIi  AND   POSTEEIOE,   SCLEROSIS.) 

The  term  ataxic  paraplegia  seems  the  most  accurate  clinical  designa- 
tion for  a  disease  of  the  spinal  cord  which  presents  a  combination 
of  the  symptoms  of  paraplegia  and  ataxy,  and  consists  in  combined 

•  •  Deut.  Ztsch.  f.  H arvenheilk./  Bd.  vi. 
t  Ibid.,  Bd.  iv, 
J  '  Virch.  Archiv,*  cxxvi. 

§  '  Amer.  Journ.  Med.  Sci.,'  1893;  *Med.  News/  1897. 
II  'Sem.  Med.,'  1896. 
^  '  St.  Biirt.'s  Hosp.  Eeports,'  xxv. 
**  Ibid.,  xxvii. 


504  SPINAL    CORD. 

disease  of  tlie  posterior  and  lateral  columns.  Although,  its  clinical 
features  present  some  varieties,  and  may  approach  those  of  each  of  its 
constituent  forms  of  disease,  yet  in  the  majority  of  cases  the  symptoms 
are  uniform,  and  sufficiently  characteristic  to  justify  the  distinction 
of  the  disease  and  its  separate  description.  As  early  as  1867  West- 
phal  described  combined  sclerosis  of  lateral  and  posterior  columns  in 
general  paralysis.  Erb,  von  Leube,  Prevost,  Pierret,  and  others, 
during  the  next  ten  years,  published  cases  definitely  showing  the 
simultaneous  occurrence  of  sclerotic  changes  in  the  lateral  and  pos- 
terior columns.  Yon  Ley  den,  in  his  book  on  spinal  cord  diseases  in 
1875,  briefly  referred  to  the  condition  as  one  of  "  combined  sclerosis 
of  the  posterior  and  lateral  columns,"  and  mentioned  a  case  which  he 
had  seen.  The  first  systematic  description  of  the  condition,  however, 
was  that  given  in  1877  by  Kahler  and  Pick  in  an  article  on  "  Com- 
bined System  Affection  of  the  Spinal  Cord."  C.  Westphal  next  pub- 
lislied  a  paper  on  "  Combined  Affection  of  the  Tracts  of  the  Spinal 
Cord,"  in  which  five  cases  were  described.  Later  Dana,  in  America, 
described  similar  cases ;  Grasset,  going  on  clinical  features,  described 
a  similar  condition  as  "  ataxo-spastic  tabes  "  and  Dejerine  as  "  ataxo- 
paraplegic  tabes."*  In  the  first  edition  of  this  book,  published  in 
1886,  a  condition,  similar  both  clinically  and  pathologically,  of  which 
the  writer  had  seen  several  cases,  was  described  under  the  name  of 
ataxic  paraplegia,  a  descriptive  title  indicating  the  combination  of 
paraplegic  weakness  with  ataxy. 

Causes. — Neurotic  heredity  is  to  be  traced  only  in  a  small  propor- 
tion of  the  cases — about  one  tenth.  A  history  of  syphilis  is  as  rare 
as  it  is  frequent  in  pure  tabes.  Males  suffer  much  more  frequently 
than  females.  The  disease  usually  commences  between  thirty  and 
forty,  but  I  have  known  it  to  begin  as  early  as  nineteen  and  as  late 
as  fifty -two,  while  commencement  at  fifteen  f  and  at  sixty-one  J  is  on 
record.  Exposure  to  cold  is  to  be  traced  occasionally  as  an  exciting 
cause.  In  one  young  lady  the  symptoms  commenced  after  a  season 
of  balls,  at  which  when  heated  she  would  habitually  sit  at  open 
windows,  and  often  sleep  in  clothes  saturated  with  perspiration.  In 
a  few  cases,  severe  exertion,  such  as  excessive  athletics,  has  been  the 
apparent  cause.  A  severe  concussion  of  the  spine  has  sometimes  pre- 
ceded the  first  symptoms  by  a  few  months.  The  disease  may  also 
follow  great  sexual  excess.  In  many  cases  no  cause  can  be  tiaced, 
immediate  or  remote.  Its  general  etiology  thus  resembles  that  of 
simple  spastic  paraplegia. 

*  See  Prevost, '  Arch,  de  Physiologie,'  t.  iv ;  Pierret,  ib.  j  Babesieu,  •  Virchow's 
Arcbiv,'  Bd.  Ixxvi ;  Kahler  and  Pick,  '  Arch,  f .  Psychiatrie,'  Bd.  viii ;  Westphal,  ib., 
Bd,  viii  and  x  j  Dana,  '  New  York  Med.  Eecoid,'  July  2, 1887  ;  and  Clarke, '  Brain,* 
1890.  As  to  Dejerine's  description  see  Ladame,  'Brain,'  1890,  p.  530;  Grasset, 
'  Maladies  du  Systeme  nerveux ;'  Montpellier,  1894. 

t  Oppenheim,  '  Neur.  Cent.,*  1888,  p.  647.  Such  early  eases  are  generally 
isolated  forms  of  "  Hereditary  Ataxy." 

J  Suckling,  'Lancet,'  1886. 


ATAXIC    PAEAPLEGIA.  505 

Symptoms. — The  onset  of  the  disease  is  usually  slow  and  gradual, 
two  or  three  years  passing  before  walking  power  is  much  impaired;  it 
is  rarely  subacute,  so  as  to  reach  a  considerable  degree  in  two  or  three 
months.  The  early  symptoms  resemble  those  of  spastic  paraplegia, 
with  the  addition  of  ataxic  Tinsteadiness,  which  may  be  at  first  the 
most  prominent  symptom.  The  legs  suffer  first,  often  alone,  or  the 
arms  may  also  be  involved.  The  patient  finds  that  he  tires  more 
readily  than  before,  and  also  becomes  unsteady  on  turning  or  waiting 
in  the  dark,  and  these  symptoms  gradually  increase.  The  weakness 
may  be  slight,  but  if  all  the  movements  are  tested,  a  defect  in  power 
will  be  found  in  some,  often  most  in  the  flexors  of  the  knee  and  hip, 
and  frequently  more  in  one  leg  than  the  other.  The  patient  is  unsteady 
when  he  stands  with  feet  together,  and  he  tends  to  fall  if  the  eyes 
are  then  closed.  If  the  feet  are  bare,  the  irregular  action  of  the 
muscles  is  shown  by  the  movement  of  the  tendons  on  the  dorsum  of 
the  foot,  as  in  tabes.  In  early  cases,  the  inco- ordination  is  revealed 
by  the  patient's  gait,  which  is  distinctly  unsteady.  There  is  rarely 
the  high  movement  and  sudden  descent  of  the  feet  often  seen  in 
tabes,  but  (as  in  many  cases  of  tabes)  the  patient  is  unsteady,  reels 
on  turning,  and  has  often  to  bring  his  foot  suddenly  to  the  ground  to 
maintain  his  equilibrium.  He  may  even  have  to  steady  himself  with 
a  stick,  or  to  catch  hold  of  some  adjacent  object,  to  save  himself  from 
falling.  The  ataxy  is  equally  evident  when  he  lies,  and  attempts,  his 
eyes  being  closed,  to  touch  some  object  with  his  foot. 

The  sensory  and  reflex  symptoms  present  a  marked  contrast  to  those 
of  tabes.  Lightning  pains  are  almost  always  absent ;  I  have  only  met 
with  them  in  one  case,  and  in  this  they  were  a  transient  symptom. 
Sometimes  there  is  a  slight  dull  pain  in  the  legs,  felt  especially  on 
fatigue.  Dull  pain  in  the  sacral  region  or  in  the  spine  is  not 
uncommon,  and  is  often  an  early  symptom.  The  sacral  pain,  indeed, 
is  suflB.ciently  frequent  to  deserve  special  note.  A  girdle-pain  is  met 
with  only  in  rare  cases.  As  a  rule  there  is  no  loss  of  sensation  either 
on  the  legs  or  trunk.  Hyperaesthesia  is  equally  rare.  Reflex  action 
from  the  sole  may  be  normal  or  increased;  less  commonly  it  is 
diminished.  The  cremasteric  and  abdominal  reflexes  are  sometimes 
lost.  The  most  striking  difference  from  tabes,  however,  is  in  the  con- 
dition of  myotatic  irritability,  which,  in  the  vast  majority  of  cases,  is 
greatly  increased.  The  knee-jerk  is  quick  and  extensive ;  it  can  be 
obtained  from  above  (see  p.  22),  and  there  is  generally  a  distinct 
rectus-clonus.  The  fopt-clonus  is  also  commonly  to  be  obtained.  This 
myotatic  excess  persists  and  increases,  and  when  the  arms  are  involved 
they  present  symptoms  similar  to  those  in  the  legs — inco-ordination, 
weakness,  and  marked  excess  of  the  myotatic  irritability. 

There  may  be  conspicuous  ataxy  of  the  hands,  and  a  tendency  to 
cramp-hke  spasm  on  an  attempt  to  use  them.  The  muscles  in  both 
arms  and  legs  are  usually  well  nourished. 

Sexual  power  is  often  lost  early  in  the  disease.    The  sphincters  may 


506  SPINAL    COED. 

be  impaired,  sometimes  early,  "but  ttey  also  often  escape.  As  in  other 
diseases,  inability  to  empty  the  bladder  is  apt  to  develop  insidiously, 
without  the  patient's  knowledge,  and  the  organ  may  thus  become 
habitually  distended. 

The  iris  usually  acts  to  light,  but  I  hare  seen  loss  of  the  light-reflex 
in  two  or  three  cases,  and  have  once  known  accommodation  to  be  lost, 
the  action  to  light  V^eing  normal.  These  symptoms  occur  chiefly  in 
cases  following  syphilis.  Optic  nerve  atrophy  occurs  only  in  rare 
oases,  far  less  frequently  than  in  tabes ;  I  have  seen  one  case  in  which 
there  were  indications  of  a  retro-ocular  axial  neuritis.  The  external 
ocular  muscles  are  unaffected  as  a  rule,  except  for  nystagmus,  which 
often  exists  on  movement  of  the  eyes,  but  seldom  when  they  are  at 
rest. 

Slight  impairment  of  articulation  is  not  uncommon;  sometimes 
there  are  irregular  tremulous  movements  of  the  face  resembling  those 
of  general  paralysis,  and  this  in  cases  in  which  there  is  no  mental 
change.  Westphal  has  observed  marked  ataxy  of  the  facial  muscles. 
As  a  rule  the  mental  state  is  either  normal,  or  there  is  merely  slight 
failure  of  memory. 

As  the  disease  increases,  the  muscular  weakness  and  reflex  spasm 
become  more  and  more  considerable ;  while  the  ataxy,  after  reaching  a 
certain  degree  (not  sufficient  to  prevent  locomotion),  necessarily  sinks^ 
into  the  background  as  the  paralysis  increases.  With  the  increased 
weakness,  the  aspect  of  the  patient  comes  to  be  that  of  spastic 
paraplegia,  described  in  the  last  section.  Indications  of  unsteadiness 
may  still  be  observed  in  isolated  movements.  The  arms,  if  previously 
free,  may  begin  to  suffer,  but  sometimes  they  escape  altogether.  The 
motor  weakness  may  go  on  to  complete  paralysis.  This  is  quickly 
reached  in  some  cases,  but  so  slow  is  the  usual  progress  of  the  disease 
that  one  patient,  in  whom  the  disease  was  at  no  time  absolutely 
stationary,  was  still  able  to  stand,  after  eight  years.  In  spite  of  the 
progress  of  the  weakness,  sensation  remains  unimpaired,  and  the 
cranial  nerves  do  not  suffer. 

The  symptoms  present,  in  some  cases,  variations  from  this  type. 
Sensation  on  the  legs  may  be  impaired,  and  the  knee-jerk  may  be  lost. 
These  cases  are,  however,  very  rare,  and  are  probably  cases  of  true 
tabes  with  lateral  sclerosis  added.  Other  cases  occur  in  which  a  girdle- 
pain,  impaired  sensation,  &c.,  suggest  extensive  changes  in  the  cord,, 
although  the  state  of  the  legs  is  that  common  in  the  disease.  Such 
cases,  as  we  shall  see,  are  probably  examples  of  chronic  myelitis,  or  are- 
intermediate  between  that  and  the  disease  now  imder  consideration. 
Ataxic  paraplegia  has  little  tendency  to  cause  death.  Indeed,  the 
fatal  cases  have,  for  the  most  part,  been  untypical,  and  do  not  convey 
an  accurate  idea  of  the  characters  of  the  disease.  The  chief  danger 
to  life  is  from  the  accidents  common  to  all  chronic  spinal  affections, 
— bedsores,  and  especially  kidney  disease,  from  undiscovered  or 
untreated  imperfect  action  of  the  bladder. 


ATAXIC   PARAPLEGIA.  507 

Among  complications  the  most  important  are  mental  changes 
resembling  those  of  general  paralysis  of  the  insane,  of  which  indeed 
this  combined  sclerosis  may  form  part.  Slight  muscular  atrophy 
sometimes  occurs.  Arthritis  of  doubtful  significance  has  been  ob- 
served. A  patient  of  Westphal's  presented  derangement  of  the 
sympathetic,  and  died  from  peculiar  spasm  of  the  muscles  of  respira- 
tion.    Visceral  crises,  however,  are  practically  unknown. 

Pathological  Anatomy. — In  all  cases,  the  spinal  cord  has  pre- 
sented sclerosis  of  both  posterior  and  lateral  columns  ;  but  the  precise 
extent  and  degree  of  the  degeneration  are  subject  to  considerable 
variations.  As  a  general  rule,  the  sclerosis  of  the  posterior  columns 
differs  from  that  of  tabes  in  two  particulars.  First,  it  is  not  more 
intense,  and  often  it  is  less  intense,  in  the  lumbar  than  in  the  dorsal 
region  of  the  cord.  Sometimes,  indeed,  as  in  the  case  shown  in 
Fig.  135,  in  the  middle  and  lower  parts  of  the  lumbar  region,  the 
posterior  columns  may  be  free  from  sclerosis,  although  it  is  considerable 
in  the  dorsal  region  and  at  the  junction  of  this  with  the  lumbar 
enlargement  (b)  .  The  second  difference  is  that  the  sclerosis  has  not 
that  special  mtensity  in  the  root-zone  of  the  postero-external  column 
which  characterises  the  lesion  of  tabes.  In  rare  cases  the  whole 
posterior  column  in  the  lumbar  region  is  diseased.  The  part  of  the 
external  column  near  the  commissure  and  near  the  neck  of  the  posterior 
horn  usually  remains  free.  Sometimes  the  degeneration  does  not 
extend  up  to  the  posterior  surface  of  the  cord ;  for  instance,  as  in  the 
case  figured,  it  affects  chiefly  the  middle  three  fifths  of  the  posterior 
columns.  When  the  degeneration  is  considerable,  the  posterior 
median  columns,  in  the  upper  part  of  the  cord,  may  present  the  usual 
ascending  degeneration  of  secondary  origin.  When  the  lesion  is  slight 
in  degree  in  the  lower  half  of  the  cord,  there  may  be  only  a  diffuse 
degeneration  of  the  columns  in  the  cervical  region,  similar  to  that 
below,  and  not  the  more  intense  limited  affection  of  the  median  part 
which  occurs  when  there  is  a  typical  ascending  degeneration  from  a 
considerable  lesion  of  the  column  lower  down. 

The  degeneration  in  the  lateral  columns  is  also  variable  in  extent 
and  position,  and  is  often  not  strictly  "  systemic"  in  character,  i.  e.  is 
not  strictly  limited  to  a  single  system  of  fibres,  although  the  pyra- 
midal tracts  are  chiefly  affected.  In  one  or  two  cases,  indeed,  the 
whole  pyramidal  tract,  and  this  alone,  has  been  degenerated  ;  but 
more  often  the  sclerosis,  while  intense  in  this  tract,  also  extends  in 
front  of  it  into  the  mixed  zone  of  the  lateral  columns  (Fig.  135,  a,  b). 
A  similar  extension,  however,  is  usually  met  with  in  the  sclerosis  that 
attends  degeneration  of  the  anterior  cornua ;  compare  Fig.  133.  The 
lateral  limiting  layer,  between  the  pyramidal  tract  and  the  grey  matter, 
may  be  also  invaded.  The  direct  cerebellar  tract  often  escapes,  as  in 
Fig.  135  A  (b  is  below  its  level  of  origin),  but  it  is  affected  in  some  cases. 
Occasionally  a  zone  of  sclerosis  has  existed  in  the  whole  periphery  of 
the  cord,  extending  deeply  into  the  lateral  column  in  the  position  of  the 


508 


SPINAL    COUD. 


pyramidal  tract.  Tliis  tract  has 
been  found  diseased,  in  some 
degree,  in  all  cases,  and  in 
several  the  extent  of  its  degene- 
ration has  been  greater  in  the 
lower  part  of  the  cord  than  in 
the  tipper.  The  direct  (anterior) 
tract  may  be  affected  as  well  as 
the  lateral,  and  is  commonly  in- 
volved when  the  lesion  is  consi- 
siderable  in  the  upper  part  of 
the  cord.  As  in  most  other  de- 
generative diseases  of  the  cord, 
there  may  be  some  increase  of 
connective  tissue  in  the  un- 
affected columns.  Asa  rule  no 
morbid  change  has  been  recog- 
nised in  the  grey  matter  or  in 
the  membranes.  In  one  case 
only  was  there  slight  meningitis. 
Disease  of  the  ascending  root  of 
the  fifth  has  been  found  in  some 
cases  ;*  in  one  there  was  also 
atrophy  of  the  cells  of  the  oculo- 
motor nucleus,  and  the  case 
thus  differed  considerably  from 
the  type.  The  muscles  have 
been  found  normal. 

Pathology. — The  double  le- 
sion in  the  posterior  and  lateral 
columns  supplies  an  explanation 
of  the  two  sets  of  symptoms 
which  characterise  the  disease. 
Fig.  135.— Ataxic  paraplegia,  combined  The  inco-ordination  must  be  re- 
lateral  and  posterior  sclerosis.  A,  upper  J,  T  J.  J.T.  J-  i:  j-i,„ 
dorsal;  B,  last  dorsal;  C,  mid-lumbar.  furred  to  the  disease  ot  the  pos- 
The  posterior  columns  are  free  from  terior  columns,  which,  greatest 
sclerosis  in  C,  except  in  their  anterior 
parts;   in  B    the   disease   involves  the 

middle  and  anterior  parts  of  both  post.-med.  and  post.-ext.  columns  except  in  the 
neighbourhood  of  the  neck  of  the  horn  ;  in  A  the  sclerosis  is  slighter  and  is  con- 
fined to  the  middle  three  fifths  of  these  columns.  The  sclerosis  of  the  lateral 
columns  in  C  is  limited,  on  the  right,  to  the  pyramidal  tract,  on  the  left  it  extends 
in  front  of  this ;  in  B  it  is  very  dense  in  the  whole  lateral  column,  involving  not 
only  the  pyramidal  tract  but  the  limiting  layer,  and  part  of  the  "  mixed  zone ;"  in 
A  it  is  similar  in  extent  but  slighter  in  degree.  There  is  some  increase  of  tissue 
throughout  the  anterior  columns,  and  a  focus  of  sclerosis  near  the  anterior  fissure, 
on  the  right  in  A  and  B,  on  both  sides  in  C.f 


•  Oppenheim,  «Neur.  Cent.,'  1888,  p.  647. 

t  I  am  indebted  to  Mr.  J.  Hopkins  for  the  opportunity  of  drawing  these  sections. 
The  patient  was  a  man  aged  twenty-one,  in  whom  weakness  of  the  legs  commenced 


ATAXIC  PARAPLEGIA.  509 

in  tlie  dorsal  region,  involves  there  the  fibres  that  conduct  impulses 
from  the  muscles,  probably  to  the  cerebellum.*  The  effect  must  be 
to  lessen  the  cerebellar  guidance,  and  this  explains  the  resemblance 
of  the  inco-ordination  to  that  in  cerebellar  disease.  The  fact  that 
the  disease  does  not  extend  into  the  lumbar  root-zone  accounts  for 
the  integrity  of  the  muscle-reflex  action,  and  enables  us  to  understand 
this  marked  difference  from  tabes.  Disease  of  the  direct  cerebellar 
tract  probably  has  an  influence  similar  to  that,  of  the  posterior 
median  columns.  That  interruption  of  the  conducting  path  in 
the  cord  will  produce  inco-ordination  is,  as  we  have  seen  (p.  258), 
well  established.  The  sclerosis  in  ataxic  paraplegia  occupies  also  the 
region  of  the  cord  in  which  the  short  vertical  fibres  run,  connecting 
the  posberior  grey  matter  at  different  levels,  and  it  is  possible  that 
the  damage  to  these  fibres  may  contribute  to  the  ataxy. 

The  only  recognisable  lesion  that  can  be  regarded  as  the  cause  of 
the  paralysis  is  the  degeneration  of  the  pyramidal  tract.  The  state- 
ments made  regarding  spastic  paraplegia  apply  also  to  the  disease,  and 
need  not  be  repeated.  The  lesion  has  been  found  greater  on  the  side 
on  which  one  leg  was  weaker  than  the  other.  The  variations  in  the 
amount  of  disease,  which  does  not  always  correspond  to  the  degree  of 
palsy,  are  probably  due  to  the  fact  that  the  latter  may  be  caused  by 
degeneration  of  the  termination  of  the  fibres,  which  may  be  much 
greater  than  that  of  the  fibres  higher  up  the  cord.  In  most  cases  that 
have  been  examined,  the  visible  disease  was  greater  in  the  lower  than 
in  the  upper  part  of  the  cord,  increasing  from  above  downwards,  a 
fact  which  suggests  that  the  maximum  lesion  is  at  the  lowest  part — 
in  the  terminal  ramification  within  the  grey  matter. 

The  chief  pathological  difficulty  arises  from  the  fact,  which  we  have 
already  considered  in  connection  with  spastic  paraplegia,  that  the 
morbid  process  often  extends  beyond  the  limit  of  the  tracts  chiefly 
diseased.     It  tends  to  assume  a  diffuse  character,  so  that  it  has 

at  twenty,  after  a  wetting,  improved,  and  then  slowly  increased.  He  was  admitted 
a  year  after  the  onset,  with  considerable  weakness  of  the  legs,  a  reeling,  unsteady 
gait,  increased  knee-jerk,  foot-clonns,  but  with  no  anaesthesia,  wasting  of  muscles, 
pains,  or  affection  of  the  arms.  The  symptoms  slowly  increased,  paraplegia  became 
absolute,  and  the  spasm  very  intense,  sometimes  flexor  and  sometimes  extensor. 
There  was  a  doubtful  impairment  of  sensibility  to  touch  on  the  legs,  but  no  loss  to 
pain.  The  sphincters  became  affected,  bedsores  formed,  and  the  patient  died  two 
years  after  the  onset.  Fuller  details  will  be  found  in  'Brain,'  October,  1883, 
p.  383. 

*  Much  evidence  has  been  obtained  of  the  connection  of  the  posterior  median 
columns  with  the  cerebellum  through  the  grey  matter  of  its  bulbar  nucleus,  and 
also  of  the  effect  of  interruption  of  these  columns  in  the  dorsal  cord,  in  causing 
inco-ordination  like  that  of  cerebellar  disease.  Besides  the  experiments  of  Bech- 
terew  on  the  latter  point  already  mentioned  (p.  215),  a  recent  important  investiga- 
tion into  the  connections  of  the  cerebellum  fully  confirms  that  with  the  post.- 
pyramidal  nucleus  (Brosset,  'Contrib.  k  I'etude  des  Connexions  du  Cervelet,'  Paris, 
1891). 


510  SPINAL    COED. 

been  regarded  by  some  as  rather  a  clironic  myelitis  than  a  system 
degeneration.  It  may  occupy  an  intermediate  position,  and  illustrate 
the  fact  that  there  is  a  close  connection  between  the  primary  decay  of 
nerve -elements  and  the  growth  of  the  connective-tissue  elements  that 
replace  the  former.  In  a  pure  secondary  degeneration  this  growth  is 
limited  to  the  tract  affected,  bat  even  in  tabes  it  may  manifest  some 
degree  of  independence,  which  seems  to  be  still  greater  in  ataxic 
paraplegia.  Sometimes,  indeed,  the  primary  morbid  tendency  may  be 
double,  involving  both  the  nerve-elements  and  interstitial  tissue.  If 
it  so  far  preponderates  in  the  latter  as  to  cause  considerable  changes 
outside  the  tracts  specially  diseased,  and  marked  symptoms,  such  as 
a  girdle-pain  or  loss  of  sensation,  the  condition  is  really  one  of  chronic 
myelitis,  and  the  cases  should  be  so  classified.  This  is  probably  the 
true  solution  of  the  problem,  which  has  been  much  discussed,  whether 
the  malady  is  or  is  not  a  system  disease.  It  is  so  in  typical  cases,  but 
it  is  on  the  border  of  the  group  of  system  diseases,  and  intermediate 
cases  connect  it  with  diseases  of  diffuse  character  and  non-systemic 
nature. 

Diagnosis. — The  diseases  from  which  ataxic  paraplegia  has  to  be 
distinguished  differ  according  to  the  stage  of  the  disease  at  which  the 
diagnostic  problem  presents  itself.  The  dominant  symptom  in  the 
early  period  is  the  in  co-ordination ;  in  the  later  period,  the  spastic 
palsy.  Hence  the  disease  in  the  early  stage  is  liable  to  be  confounded 
with  pure  locomotor  ataxy,  but  the  condition  of  the  knee-jerk,  lost  in 
the  one,  excessive  in  the  other,  is  distinctive.  In  the  rare  cases  of 
early  tabes  in  which  it  is  not  lost,  it  is  never  increased.  In  tabes, 
there  is  not  the  loss  of  power  which  will  always  be  found  in  ataxic 
paraplegia,  and  when  a  clonus  and  extensor  spasm  are  added,  there 
should  be  no  risk  of  confusion ;  but  it  is  necessary  to  emphasise  the 
distinction,  because  cases  with  much  unsteadiness  are  frequently 
described  as  tabes  with  preserved  knee-jerk.  From  primary  spastic 
paraplegia  the  diagnosis  depends  on  the  presence  of  inco-ordination, 
or  in  a  history  of  it,  if  voluntary  power  has  become  too  slight  to  be 
susceptible  of  derangement.  Ataxic  paraplegia  is  spastic  paraplegia 
plus  inco-ordination.  The  so-called  "hereditary  ataxy"  presents  a 
close  resemblance  to  ataxic  paraplegia,  and  is,  indeed,  as  we  shall 
see,  intermediate  between  the  disease  and  true  tabes.  It  is  distin- 
guished by  its  occurrence  in  several  members  of  the  same  family,  and 
by  the  common  loss  of  the  knee-jerk ;  while  the  presence  of  nystag- 
mus, of  some  impairment  of  articulation,  and  of  other  symptoms 
described  in  the  account  of  the  disease,  distinguish  the  isolated  cases 
that  are  sometimes  met  with.* 

The  distinction  from  diffuse  or  focal  chronic  myelitis  depends  chiefly 
on  the  extent  of  the  symptoms  in  the  former,  and  their  limitation  ia 

*  The  condition  closely  resembles  that  met  with  as  a  result  of  toxic  iufliience 
pvobably,  in  conditions  of  anaemia,  debility,  &c.  Some  of  the  cases  described  aa 
ataxic  paraplfgia  have  doubtless  been  of  this  nature  (see  next  section). 


SCLEROSIS    FROM    TOXIO   BLOOD-STATES.  511 

the  latter.  The  subacute  forms  of  myelitis  are  also  distinguished  by 
their  more  rapid  onset.*  The  greatest  difficulty  is  presented  by  cases 
which  have  a  subacute  onset,  and  the  distinction  from  a  local  myelitis 
involving  the  posterior  and  lateral  columns  may  be  very  difficult.  It 
depends  chiefly  on  the  progressive  tendency  of  the  symptoms,  which 
contrasts  with  the  regressive  tendency  of  myelitis.  It  is  possible  that 
when  there  is  a  degenerative  tendency,  a  focal  myelitis  may  set  up  a 
jirogressive  degeneration  in  the  columns  concerned,  as  it  certainly  may 
set  up  a  degeneration  limited  to  the  posterior  columns. 

A  tumour  in  the  middle  lobe  of  the  cerebellum  may  cause  unsteadi- 
ness, closely  resembling  that  of  some  cases  of  ataxic  paraplegia,  and 
it  may  also  cause  weakness  of  the  legs  with  increased  knee-jerk,  from 
the  pressure  on  the  pyramidal  fibres  as  they  pass  through  the  pons. 
In  such  cases,  indeed,  we  probably  have  the  two  elements  of  the 
disease,  produced  in  a  different  manner,  by  lesions  of  different  posi- 
tion. We  have  a  degeneration  of  the  lateral  columns  which  is  secon- 
dary instead  of  primary,  and  we  have  disease  of  the  co-ordinating 
centre  instead  of  interruption  of  the  path  to  it.  But  the  weakness  in 
the  legs  is  never  great  in  cerebellar  tumour,  and  special  symptoms  of 
this  are  never  absent.  Occipital  headache,  vomiting,  and  optic  neuritis 
are  present  in  most  cases.  In  a  case  in  which  the  pons  is  compressed, 
some  cranial  nei-ves  are  usually  also  damaged. 

Prog-nosis. — The  prognosis  in  ataxic  paraplegia  is  similar  to  that 
in  the  spastic  form,  and  depends  on  similar  considerations,  which  need 
not  be  here  repeated. 

Treatment. — The  treatment  of  ataxic  paraplegia  is  the  same  as  that 
of  the  allied  diseases,  and  especially  of  spastic  paraplegia,  to  the  account 
of  which  the  reader  is  referred. 


SCLEROSIS    OF   THE    CORD   FROM   TOXIC   BLOOD- 
STATES. 

Pellagra. 

Pellagra  is  an  endemic  malady  which,  though  fortunately  unknown 
in  this  country,  deserves  mention  here  on  account  of  the  incidence 
of  its  effects  on  the  spinal  cord.  Its  chief  anatomical  lesion  is  a 
degeneration  of  the  lateral  and  posterior  colunms  of  the  cord,  corre- 
sponding closely  to  that  of  ataxic  paraplegia,  and,  as  in  that  disease, 
with  a  greater  affection  of  the  lateral  than  of  the  posterior  columns. 
It  differs,  however,  ia  the  fact  that  some  atrophy  of  the  large  nerve- 
cells  of  the  anterior  comua  is  common,  and  also  in  the  constancy  with 

*  For  some  instructive  notes  ou  this  point  in  diagnosis  see  Drescbfeld,  'Brain,' 
January,  1888. 


512  SPINAL    CORD. 

whicli  chronic  inflammation  of  the  pia  mater  is  met  "with,  sometimes 
accompanied  bj  the  formation  of  bony  plates  in  the  arachnoid. 

The  Cause  of  the  disease  appears  to  be  the  action,  on  the  elements 
of  the  spinal  corcl,  of  an  organised  virus  (or  of  some  product  of  this) 
which  is  taken  into  the  system  with  diseased  or  unripe  maize.     Even 

spirit  distilled  from  such  unripe  maize 

i^^n^S^^     _^~~.  ^'-^Q        ^^y  cause   the   disease.     The   distri- 

cy^^^-'-    "    '  '  ~^-~-^  bution  of  the  disease  in  the  north  of 

Italy,  where  the  maize  is  frequently 
gathered  unripe,  is  significant.  The 
virus  has  been  thought  by  some  (as 
Lombroso)  to  be  a  fungus  growing 
in  the  maize  ;  by  others  to  be  specific 
micro-organisms  of  other  than  fun- 
goid nature,  present  in  the  maize, 
which  develop  in  the  system  and  pro- 
duce there  the  toxic  agent  that  has  a 
special  action  on  the  nerve-elements 
(Belmondo).  The  researches  of  Tuc- 
zek*  have  clearly  shown  that  the 
change  is  a  sclerosis  of  the  cord  de- 
pendent upon  some  toxic  substance 
occurring  in  diseased  or  unripe  maize. 
\5  ^  i  y.J'  The  effects  of  the  poisons  fall,  not  on 

"  "^  ^"^"^  the    peripheral  nervous   system,    but 

on  the  spinal  cord,  and,  as  is  usual  in 
such  conditions  (see  previous  section), 
the  sclerosis  induced  is  symmetrical, 
and,  as  will  be  seen  from  the  illus- 
trations (Fig.  136),  in  its  distribution 
is  very  similar  to  that  met  with  in 
conditions  of  anaemia  and  debility 
(p.  513). 

The  Symptoms  resemble  very  closelv 
Fig-.     136. — Sclerosis    in    pellagra    ,,  <•       -      •  i      •  mi      '' 

(Tucz.k).     The   darkly    shaded   ^^^o^e    of    ataxic    paraplegia.  ^  There 
areas  indicate  the  sclerosis.  is    weakness   in  the    legs,    with    in- 

creased myotatic  irritability  going  on 
to  spasm ;  unsteadiness  of  movement,  especially  in  the  early  stages, 
sometimes  accompanied  by  tremor.  Sensibility  may  be  normal, 
or  the  perception  of  touch  or  pain  may  be  increased  or  lessened. 
Belmondo,  from  an  important  series  of  investigation s,t  has  arrived  at 
the  conclusion  that  the  symptoms  depend  on  a  primary  systemic 
degeneration  of  the  nerve-elements  of  the  pyramidal  tracts  and 
posterior  columns,  determined  by  the  influence  of  the  toxic  agent. 
The  course  of  the  disease  is  chronic,  but  sometimes  an  intensely  acute 

*  Tnczek,  *  Klinische  u.  Anatomische  Studien  iiber  die  Pellagra,'  Berlin,  1893, 
t  •  Riv.  Sporiment.,'  1889-90,  xv  and  xvi. 


SCLEROSIS  FROM  TOXIC    ELOOD-STATES.  513 

stage  comes  on,  -with,  symptoms  of  spinal  meningitis,— a  condition  to 
which  the  term  "  typhus  pellagrosus  "  has  been  applied.  After  death 
in  this  state  there  are  often  found  commencing  acute  myelitis,  in 
addition  to  the  inflammation  of  the  membranes,  and  also  swelKng  of 
the  intestinal  lymphatic  glands,  with  signs  of  enteritis, — indications 
of  an  acute  blood-state  which  justify  the  view  that  the  affection  is, 
primanlj,  a  toxsemic  malady. 


SciiEEOSIS  OF   THE   COED   EBSTJIiTING  FEOM   OTHEE   TOSIG   BlOOD- 

STATES. 

During  the  last  few  years  the  effects  of  toxic  blood-states  in  causing 
changes  in  different  parts  of  the  nervous  system  have  been  widely 
recognised.  The  occasional  occurrence  of  sclerotic  changes  in  the 
spinal  cord  in  association  with  alcoholic  neuritis,  not  merely  as  a 
result  of  the  neuritis,  has  been  already  referred  to  in  the  chapter  on 
Multiple  Neuritis,  but  the  researches  of  Williamson  *  have  proved  the 
occasional  occurrence  of  sclerosis  of  the  posterior  columns  in  diabetes 
— a  disease  with  a  well-recognised  toxic  blood-state. 

In  1887  Lichtheimf  described  three  cases  of  pernicious  anaemia 
which,  in  addition  to  the  usual  symptoms  of  that  disease,  had  sym- 
ptoms of  spinal  cord  affection.  In  two  the  symptoms  pointed  to  a 
mixed  affection  of  lateral  and  posterior  columns,  in  the  other  the 
posterior  columns  were  thought  to  be  chiefly  affected.  In  the  last 
case  no  post-mortem  examination  was  obtained,  but  in  the  other  two 
distinct  spinal  cord  changes  were  found,  viz.  almost  complete  degene- 
ration of  the  columns  of  G-oll,  and  similar  but  slighter  affection  of 
the  pyramidal  tracts.  Lichtheim  suggested  a  toxic  blood-state  as  the 
probable  cause  of  these  changes.  Minnich,J  a  pupil  of  Lichtheim, 
published  a  series  of  similar  cases,  and  supplemented  his  work  by 
examining  the  cords  of  five  patients  with  pernicious  anaemia  who  had 
had  no  symptoms  of  spinal  cord  affection.  He  found  two  sets  of 
changes  present,  (1)  capillary  haemorrhages  with  apparently  miliary 
sclerosis  resulting  from  them,  and  (2)  a  slight  sclerotic  change  in 
certain  parts  of  the  cord  as  indicated  by  slighter  coloration  after 
hardening  in  bichromate  salts,  of  the  posterior  and  also  of  the  ante- 
rior and  lateral  columns. 

He  also  found  similar  changes  in  the  cords  of  patients  the  subjects 
of  cachectic  and  debilitating  conditions,  such  as  chronic  jaundice, 
leukaemia,  and  cerebellar  tumour.  Within  the  period  that  has  elapsed 
since  then  similar  cases   have  been  described  by  Van  lToorden,§ 

*  '  Brit.  Med,  Journ.,'  1894,  p.  398, 
t  *  Neur.  Cent.,'  1887,  p.  236. 
t  Ibid.,  1889,  p.  662. 
§  *Charite  Annalen,' 1891. 

VOL.  I.  33 


614  SPINAL    COED. 

Eisenlohr,*  ISTonne,!  Bowman,  J  James  Taylor,  §  Micliell  Clark,  || 
Eisien  Eussell,T[  and  others,  some  of  the  cases  having  the  usual 
symptoms  of  pernicious  anaemia,  others,  however,  being  merely  anaemic, 
some  in  whom  anaemia  was  not  obvious,  but  in  whom  there  were  dis- 
tinct evidences  of  debility  or  mal-nutrition.  In  1891  Putnam,  of 
Boston,**  described  "  a  group  of  system  scleroses  of  the  spinal  cord, 
associated  with  diffuse  collateral  degeneration  occurring  in  enfeebled 
persons  past  middle  life,  especially  in  women."  In  four  cases  exa- 
mined post  mortem  sclerosis  was  found  in  both  lateral  and  posterior 
columns  of  the  cord,  and  he  also,  found  disintegration  of  cells  in  the 
grey  matter.  These  cases  are  no  doubt  similar  in  origin  to  those 
described  by  Lichtheim. 

Symptoms. — In  the  majority  of  cases  the  onset  of  the  spinal  cord 
symptoms  is  usually  preceded  by  a  period  of  bad  health,  more  or  less 
prolonged,  or  by  some  condition  leading  to  impairment  of  vitality.  Thus, 
in  one  case,  a  profuse  loss  of  blood  immediately  preceded  the  anaemia, 
to  which  the  spinal  cord  affection  was  afterwards  added.  Difl&culty 
or  uncertainty  in  walking  is  first  complained  of,  and  one  leg  may  be 
affected  earlier  and  more  severely  than  the  other.  Sharp  lancinating 
pains,  suggesting  the  lightning  pains  of  tabes,  are  occasionally  present, 
and  paraesthesiae,  not  necessarily  painful,  are  frequent.  The  knee- 
jerks  are  usually  at  first  normal  or  diminished,  and  there  may  be  a 
difference  on  the  two  sides.  In  some  cases,  however,  they  are  early 
lost,  or  the  reflex  may  be  j)resent  on  one  side  and  not  on  the  other. 
When  they  are  exaggerated,  as  sometimes  happens,  and  some  ataxy  is 
present,  the  clinical  pictiu-e  presented  is  that  of  ataxic  paraplegia.  The 
weakness  gradually  increases  until  the  patient  is  unable  to  get  out  of 
bed,  then  sensory  impairment  may  occur,  considerable  rigidity  may 
come  on,  and  the  knee-jerks,  in  cases  in  which  they  were  present,  m.ay 
be  no  longer  obtainable.  The  arms  also  may  be  involved,  and  in  one 
case  curious  rhythmical  movements  at  the  wrist  and  elbow  were  pre- 
sent, suggesting  those  of  canine  chorea,  and  probably  of  significance, 
as  we  shall  see  when  we  come  to  discuss  the  morbid  anatomy.  Con- 
tractions may  be  superadded,  and  bedsores,  cystitis,  and  gradual 
exhaustion  sooner  or  later  lead  to  a  fatal  issue. 

Besides  the  symptoms  enumerated  there  may  of  course  be  the  usual 
symptoms  of  profound  anaemia — such  as  reduction  in  haemoglobin 
and  number  of  corpuscles,  enlargement  of  spleen,  and  haemorrhages  in 
the  retinae  and  elsewhere.  But,  as  has  been  stated,  obvious  anaemia 
is  not  constant  in  the  condition  under  notice. 

*  ♦  Deut.  Med.  Wochenschr.,'  1892. 
t  *  Arch,  f .  Psych.,'  xxv,  1893. 
j  'Brain,'  1894. 
§  •  Med.-Chir.  Trans.,'  1895. 
II  'Brit.  Med.  Journ.,'  1897. 
•ir  '  Lancet,'  1898. 
••  '  Journ.  of  Nerv.  and  Ment.  Diseases,'  1891. 


SCLEROSIS    FROM    TOXIO    BLOOD- STATES. 


515 


MoEBiD   Anatomy.— The  changes  found  in  the   spinal  cord  are 
suriously  uniform  (Fig.  137).     In  the  cervical  region  they  are  usually 


ftff.  187. — SLowinp:  sclerosis  occurring  in  a  case  of  profound  aneemia. 
The  sclerotic  areas  are  decolourised.  Note  in  the  lumbar  cord  the  com- 
parative escape  of  the  septo-mar^^inal  and  coma-comniiasilXiil  tracts 
A,  cervicaL     B,  dorsal.    C*  Inmbar. 


516  SPINAL   COED. 

wide-spread,  and  are  found  in  anterior,  lateral,  and  postc:L,.ur  columns; 
the  part  of  the  anterior  column  chiefly  affected,  and  also  of  tlie  lateral, 
being  tlie  pyramidal  tracts,  although  th"  change  is  not  restricted  to 
these  in  either  region.  In  the  dorsal  region  the  change  is  still  exten- 
sive, but  there  is  a  gradual  diminution  downwards,  so  that  in  the 
lumbar  region  the  sclerosis  is  much  less  wide-spread  and  less  complete 
in  character.  In  this  region  the  part  of  the  posterior  column  next  the 
commissure  is  usually  spared,  as  also  that  close  to  the  posterior  septum 
— the  septo-marginal  tract  of  probably  endogenous  fibres  already 
alluded  to  (p.  218).  It  is  well  seen  in  the  accompanying  illustration^ 
It  is  interesting  to  note  that  the  other  tract  in  the  lumbar  region  which 
is  s^Dared  probably  also  consists  of  endogenous  fibres — the  so-called 
cornu-commissural  tract.  The  sclerosis  in  the  pyramidal  tracts  in  the 
lumbar  cord  usually  becomes  restricted  to  a  small  triangular  area- 
close  to  the  posterior  root.  These  degenerations  have  not  been  found 
to  extend  upwards  beyond  the  lower  end  of  the  medulla  oblongata, 
and  although  slight  but  distinct  changes  have  been  described  in  the 
cells  of  the  anterior  horn,  and  also  in  those  of  Clark's  column,  no 
definite  evidence  of  affection  of  the  peripheral  nerves  has  been  found 
in  the  few  cases  in  which  changes  have  been  looked  for.  It  is,  however, 
more  than  probable  that  in  some  cases  such  changes  are  present,  and 
it  may  be  expected  that  they  will  be  found  when  the  nerves  are 
regularly  and  carefully  examined. 

The  change  which  constitutes  the  scleiosis  is  apparently  a  simple 
thickening  and  increase  of  the  neuroglia  replacing  the  nerve-elements 
which  have  disappeared.  There  is  also  a  marked  increase  in  the 
number  of  vessels  in  the  affected  areas,  and  this  has  been  especially 
well  seen  in  most  cases  in  which  the  change  has  been  described  in  the 
lumbar  regions.  In  one  of  Russell's  cases  "  there  was  a  perfect  net- 
work of  small  thin- walled  engorged  vessels,  in  addition  to  larger  vessels 
with  thickened  walls."  But  even  with  this  condition  there  was  no 
marked  infiltration  of  the  surrounding  tissue.  In  no  case  in  which 
these  degenerations  have  been  present  has  any  haemorrhage  been  found 
in  any  of  the  affected  tracts.  It  is  significant,  however,  that  Lichtheim 
and  Minnich  describe  in  cases  of  pernicious  anaemia,  in  which  no  sym- 
ptoms of  spinal  cord  affection  have  been  noted  during  life,  small  scle- 
■  rotic  foci  the  result  of  haemorrhages  in  different  parts  of  the  spinal 
cord,  The  importance  and  significance  of  this  wiU  be  alluded  to  in 
discussing  the  pathology  of  the  condition  under  consideration. 

Pathology. — There  seems  little  reason  to  doubt  that  the  sclerotic 
condition  present  in  these  cases  is  the  result  of  a  toxic  condition  of 
the  blood.  Its  origin  under  conditions  of  at  least  mal-nutrition  and 
debility,  its  presence  in  some  patients  who  are  undoubtedly  suffering 
from  pernicious  anaemia,  and  the  occurrence  of  shghter  changes  of 
similar  distribution  in  conditions  like  leukaemia,*  all  poiat  to  the  same 
probability.     The  general  symmetry  of  its  distribution  is  also  another 

*  Muller,  *  Inaugural  Dissertation/  Berlin,  1895. 


SCLEROSIS    FROM    TOXIC    BLOOD-STATES.  517 

indication  that  the  blood  is  probably  the  vehicle  by  which  the  materies 
morbi  is  brought  to  its  destination  ;  and  it  is  very  significant  that  the 
•changes  in  the  spinal  cord  in  pellagra,  an  admittedly  toxic  disease,  are 
practically  identical  in  distribution  with  those  in  the  conditions  under 
notice.  The  nature  of  the  poison  we  do  not  know,  and  it  cannot  be 
said  with  certainty  whether  it  acts  directly  on  the  nervous  elements, 
-or  by  first  producing  disease  of  the  blood-vessels.  In  many  cases  the 
blood-vessel  walls  are  found  thickened,  in  some  instances  to  the  point 
of  occlusion,  and  the  occurrence  of  haemorrhages  in  the  retinse,  and 
elsewhere  under  similar  conditions,  suggests  that  diseased  vessels  and 
consequently  altered  blood-supply  may  give  rise  to  interference  with 
"the  nutrition  of  the  cord,  and  so  produce  a  sclerotic  condition.  Such 
an  explanation  is  not  probable.  Yet  there  seems  no  reason  to  doubt 
that  in  some  of  the  cases  the  occurrence  of  small  haemorrhages  in  the 
•cord,  similar  to  those  that  occur  in  the  retinae  in  pernicious  anaemia, 
may  be  an  element  in  producing  the  changes  which  we  meet  with. 
The  observations  of  Minnich  and  others  already  alluded  to,  on  the 
■cords  of  patients  suffering  from  pernicious  anaemia,  demonstrate  the 
occurrence  of  such  haemorrhages,  and  of  small  foci  of  degeneration  as 
the  result  of  them,  in  those  cases  in  which,  it  is  true,  no  special  sym- 
ptoms of  cord  affection  were  noted  during  life.  Bwt  if  such  haemor- 
rhages occur  in  these  cases,  it  seems  likely  that  they  are  not  altogether 
absent  m  cases  in  which  disease  of  the  cord  is  more  pronounced.  And 
it  must  be  confessed  that  in  some  cases  the  difference  between  two 
sections  of  the  cord,  within  a  short  distance  of  each  other,  is  greater 
than  IS  to  be  satisfactorily  accounted  for,  except  on  the  supposition  of 
some  local  condition  to  give  rise  to  the  difference.  Such  haemorrhages 
would  of  course  be  no  part  of  the  ultimate  pathology  of  the  affection, 
which,  as  we  have  said,  is  almost  certainly  toxic ;  but  they  might 
account  for  some  of  the  changes  visible  in  the  spinal  cord,  especially 
those  which  constitute  a  departure  from  true  symmetry. 

The  view  of  Nonne  and  others,  that  the  condition  is  really  one  of 
•disseminated  myelitis,  offers  many  difficulties.  The  absence  of  any 
small-celled  infiltration  such  as  is  found  in  myehtis,  the  absence  also 
of  such  ascending  and  descending  degenerations  as  foci  of  inflamma- 
tion would  produce,  seem  to  constitute  insuperable  difficulties  to  the 
a,cceptance  of  such  a  view.  That  the  condition  is  to  be  accounted  for 
by  patches  of  change,  haemorrhage  perhaps,  in  the  grey  matter,  is  most 
unlikely.  In  many  cases  no  change  is  found  in  the  grey  matter ;  when 
any  change  is  present  it  is  slight  and  non- symmetrical,  and  one  striking 
feature  is  the  escape  from  degeneration  of  the  endogenous  fibres  of  the 
cornu-commissural  and  septo-marginal  tracts  of  the  cord  (see  p.  218), 
tracts  which  connect  the  grey  matter  at  different  levels,  and  which 
would  presumably  suffer  conspicuously  in  any  primary  affection  of 
that  tissue.  So  that  on  all  grounds  the  change  is  to  be  regarded  as 
probably  of  the  nature  of  a  parenchymatous  degeneration  of  the  nerve 
substance,  with  secondary  overgrowth  and  hardening  of  the  connective 


518  SPINAL   CORD. 

tissue,  the  effect  of  some  toxic  substance  circulating  m  the  Wood, 
which  probably  also  produces  the  general  cachexia,  &c  ,  which  are 
present.  Why  the  areas  which  so  constantly  and  so  uniformly  suffer 
should  do  so  we  can  only  account  for  by  supposing  that  the  toxin 
which  causes  the  degeneration  has  some  selective  action  for  these 
parts,  just  as  the  analogous  toxin  in  tabes  has  a  special  affinity  for 
the  posterior  neurons,  or  that  these  areas  are  peculiarly  susceptible  to 
the  poison — another  way  of  stating  the  same  fact. 

Diagnosis. — The  diagnosis  in  these  cases  will  probably  become 
easier  as  we  become  more  familiar  with  them.  The  cases  differ  among 
themselres,  especially  in  regard  to  the  deep  reflexes,  so  that  the  dis- 
tinction which  has  usually  to  be  made  is  from  tabes,  toxic  peripheral 
neuritis,  lateral  sclerosis,  and  ataxic  paraplegia,  as  we  should  expect 
from  the  distribution  of  the  sclerosis,  and  there  is  little  doubt  that 
some  of  the  cases  hitherto  described  as  ataxic  paraplegia  were  of  this 
nature.*  In  the  majority  of  cases  the  closest  resemblance  is  to  ataxic 
paraplegia.  Indeed,  but  for  the  longer  course  of  the  disease  in  ataxic 
paraplegia,  and  the  absence  of  the  cachectic  condition,  and  of  the 
strong  tendency  to  death,  the  conditions  would  be  almost  identical, 
and  it  may  be  that  future  observation  may  prove  them  to  be  so.  In 
cases  in  which  the  knee-jerk  is  absent  the  condition  may  easily  be 
mistaken  for  tabes  dorsalis,  and  in  one  case  at  least  this  was  the 
diagnosis  made  until  the  post-mortem  examination  revealed  the  true 
nature  of  the  malady.  The  absence  of  characteristic  lightning  pains 
and  often  of  a  syphilitic  history,  or  of  an  early  affection  of  sphincters, 
are  points  to  be  carefully  kept  in  mind  in  deciding  whether  a  case 
belongs  to  one  or  other  class.  Peripheral  neuritis  also  offers  a  close 
resemblance  to  such  a  condition  ;  but  in  the  cases  of  this  disease  which 
we  most  frequently  meet  with  there  is  to  be  obtained  a  history  of 
indulgence  in  the  usual  toxic  cause  of  this  paralysis,  viz.  alcohol. 

Peognosis. — The  prognosis  is  necessarily  bad,  although  it  is  con- 
ceivable that  it  need  not  always  be  so.  In  one  case,  that  already 
alluded  to  as  having  been  mistaken  for  tabes,  there  was  a  history  that 
the  patient,  four  years  before  he  came  under  observation  for  the  para- 
plegia which  ultimately  proved  fatal,  had  been  in  the  same  condition, 
and  had  made  a  complete  recovery.  In  another  instance  the  patient  came 
under  observation  first  on  account  of  weakness  of  the  limbs  and 
intense  headaches.  She  was  very  anaemic,  and  under  the  influence  of 
iron  and  arsenic  she  made  apparently  a  complete  recovery,  and  went  to 
the  sea- side.  She  returned  in  six  months  in  a  condition  of  extreme 
spastic  paraplegia ;  she  became  completely  helpless,  very  anaemic, 
and  died  of  exhaustion.  She  had  marked  retinal  haemorrhages 
before  death,  and  her  spinal  cord  was  found  to  have  the  wide-spread 
sclerosis  characteristic  of  these  cases. 

Treatment. — Attention  to  general  nutrition  is,  of  course,  essential 
in  aU  such  debilitated  conditions  as  those  under  notice.  In  the 
•  See  Michell  Clark'a  case,  '  Brain,'  1890. 


»> 


"  HP]REDITARY   ATAXY  519 

definitely  anaemic  cases  iron  and  arsenic  are  clearly  indicated,  and 
they  are  so  probably  in  all  cases.  Bone  marrow  does  not  seem  as  yet 
to  have  received  a  trial,  but  that  also  in  combination  with  the  drugs 
mentioned  would  possibly  be  foxind  to  have  a  good  effect.  Cystitis, 
bedsores,  and  contractions  must  be  treated  if  they  arise.  The  pains  may 
be  severe  and  necessitate  morphia,  but  the  need  for  this  may  be  lessened 
by  injections  of  cocaine,  as  recommended  in  the  chapter  on  Tabes. 


"HEEEDITART   ATAXY" 

(feiedeeich's  disease,  heeeditaey  ataxic  paraplegia). 

The  so-called  hereditary  ataxy  is  a  form  of  ataxy,  or  rather  of  ataxic 
paraplegia,  depending  on  combined  posterior  and  lateral  sclerosis, 
which  occurs  in  families,  and  differs  further  from  the  common  forms 
of  tabes  and  of  ataxic  paraplegia  in  the  early  age  at  which  it  always 
commences,  and  in  the  presence  of  certain  additional  symptom?.  It 
is  often  termed  "  Friedreich's  disease,"  because  this  physician  first 
described  the  characteristic  features  of  the  malady  and  the  lesion  in 
the  posterior  columns.* 

The  dependence  of  the  disease  on  a  congenital  tendency  is  clearly 
shown  by  its  usual  occtirrence  in  families.  But  direct  inheritance  has 
been  traced  in  only  a  few  instances.  In  one,  the  mother  of  the  family 
affected,  and  her  mother,  both  suffered  from  the  disease.  That  a 
general  neuropathic  inheritance  is  also  sometimes  effective  is  shown, 
by  cases  in  which  there  is  a  history  of  other  neurotic  diseases,  such  as 
insanity  or  epilepsy,  in  collaterals  or  ancestors.  Two  brothers  who 
were  affected  had  another  brother  epileptic,  and  a  sister  suffered  from 
repeated  attacks  of  chorea.  Alcoholism  in  parents  has  been  supposed 
to  be  influential,  but  the  evidence  of  this  is  not  strong.  Consanguinity 
of  parents  has  existed  in  some  instances,  and  has  doubtless  intensified 
the  morbid  tendency  in  these  cases. 

The  family  tendency  of  the  disease  is  shown  by  the  affection,  in 
most  instances,  of  brothers  and  sisters.  Thus  sixty-five  cases  were 
distributed  in  nineteen  families,  giving  an  average  of  rather  more  than 
three  to  each.  The  number  affected  in  one  generation  has  varied  from 
two  to  eight.      In  the  case  in  which  the  mother  and  grandmother 

*  Friedreich's  first  account  was  given  to  a  medical  society  in  1861,  and  published 
in  'Virchow's  Archiv'  (Bde.  xxvi  and  xxvii)  in  1863.  Fnrthei- cases  were  published 
by  him  in  1876  (ib.,  Bde.  Ixviii  and  Ixx),  and  a  collection  of  57  cases  is  given  by  Dr. 
Everett  Smith, '  Boston  Med.  and  Suig.  Journal,'  Oct.  15th,  1885.  See  also  Ormerod, 
'  Brain,'  Jan.,  1888  j  Dejerine,  '  Med.  moderne,'  1890,  No.  25,  and  'La  Sem.  Med., 
1890,  Nois.  11,  12;  and  especinlly  Soca,  'These  de  Paris,'  1888  (who  has  collected 
165  cases — many,  however,  doubtful),  and  Ladame, '  Brain,'  1890,  pt.  Hi,  where  a 
full  bibliography  will  be  found;  see  also  Mackie  Whyte,  *  Brain,'  1898. 


520  SPINAL    CORD. 

Buffered,  an  tincle  and  seven  children  were  also  affected,  mating  ten  in 
one  family.  Isolated  cases  have  also  been  recorded,  and  they  seem  not 
to  be  uncommon.  Probably  the  nature  of  the  affection  has  not  been 
suspected  on  account  of  the  isolation.  In  many  of  these  cases  (to 
judge  from  personal  observations),  this  feature  extends  beyond  the 
disease ;  the  patient  is  an  only  child,  or  the  only  one  of  the  sex  in  the 
family.  Thus  one  patient  was  the  only  daughter,  and  her  four 
brothers  were  healthy.  On  the  other  hand,  it  is  often  to  be  noted 
that  the  families  in  which  cases  of  Friedreich's  disease  occur  are 
unusually  large,  as  in  one  family  of  fifteen,  in  which  three  members 
were  affected ;  another  unaffected,  however,  transmitting  the  conditions 
to  two  out  of  four  children. 

The  two  sexes  present  nearly  equal  liability;  males  slightly  pre- 
ponderate (thirty-five  males  to  thirty  females).  In  some  families  the 
two  sexes  have  suffered  equally,  but  in  others  the  disease  has  shown  a 
marked  tendency  to  affect  one  sex.  Thus  in  one  family  of  nineteen 
the  two  males  suffered,  and  the  seventeen  females  escaped.  In 
another  instance,  recorded  by  Musso,  a  brother  and  sister  (the 
offspring  of  a  melancholic  mother)  married  healthy  individuals ; 
the  brother  had  three  daughters  affected  out  of  seven  living  children ; 
and  of  the  sister's  children  three  sons  were  diseased.  A  curious  fact 
is  that  three  of  the  brother's  and  four  of  the  sister's  children  were 
born  dead. 

The  age  at  which  the  first  symptoms-  are  recognised  has  varied 
between  two  and  twenty-four  years.*  The  seventh  and  eighth  years 
of  life  are  those  in  which  disease  most  often  begins ;  and  next  com.es 
the  period  of  puberty,  from  twelve  to  sixteen.  In  isolated  instances 
the  onset  is  generally  later  than  in  the  grouped  cases.  It  begins 
somewhat  earlier  in  males  than  in  females,  and  often  commences  about 
the  same  period  in  the  same  family. 

Immediate  causes  can  rarely  be  traced ;  preceding  acute  diseases 
may  have  facilitated  the  onset,  but  can  scarcely  have  done  more. 

Symptoms. — The  first  and  chief  manifestation  of  the  disease  is  a 
gradual  impairment  of  co-ordination,  first  in  the  legs  and  afterwards 
in  the  arms.  Initial  j)ains  scarcely  ever  occur,  but  cramp  is  occa- 
sionally complained  of.  The  ataxy  is  shown  by  unsteadiness  in 
standing  and  walking,  at  first  slight,  but  slowly  increasing,  imtil  the 
feet  have  to  be  placed  wide  apart  in  standing,  and  the  patient  reels  in 
wallring  like  one  under  the  influence  of  alcohol.  The  feet  are  not 
often  raised  too  high,  unless  when  an  unusually  long  step  is  taken. 
Closure  of  the  eyes  causes  a  considerable  increase  in  the  unsteadiness 
in  most  cases ;  in  some  it  has  but  little  influence.     Children  often  first 

*  III  the  family  recorded  by  Everett  Smith,  the  father  presented  symptoms  of 
ataxic  paraplegia  at  the  age  of  sixty-six,  coming  on  gradually  after  an  attack  of 
rheumatism  produced  by  exposure  to  cold.  In  the  age  of  the  sufferer  this  case 
.stands  alone  in  the  history  of  the  disease,  and  is  not  included  in  the  figures  given. 
Tiifc  case  may  be  a  mere  coincidftuce,  or  may  show  that  a  latent  predisposition  may 
persist  through  life. 


**  HEREDITARY   ATAXY.**  521 

filio-w  the  affection  by  the  readiness  with  which  they  stumble  and  fall. 
The  impairment  of  movement  in  the  arms  is  of  a  similar  character,  but 
usually  commences  some  time  after  that  of  the  legs.  There  is  irregu- 
larity in  the  voluntary  movement  of  the  arms  and  fingers,  and  the 
ataxy  has  often  a  distinctly  jerky  character.  In  most  cases  the  power 
of  the  muscles  is  at  first  unimpaired  ;  their  nutrition  is  good,  but  the 
myotatic  irritability  is  lost.  In  most  cases  the  knee-jerk  has  been 
found  absent  as  soon  as  the  patient  came  under  observation  ;  in  one 
case  it  disappeared  after  the  oth(-r  symptoms  had  set  in  ;  in  anothei-  it 
was  normal  on  one  side,  absent  on  the  other.  In  several  there  has  been 
no  change  long  after  all  the  other  characteristic  symptoms  had  mani- 
fested themselves ;  very  rarely  it  has  been  increased,  chiefly  in  untypical 
cases. 

As  the  disease  progresses  some  jerky  irregularity  develops  in  the 
movements  of  the  neck  and  head,  so  that  the  head  presents  slight 
unsteady  movements,  sometimes  like  an  irregular  tremor,  sometimes 
closely  simulating  chorea.  Articulation  is  also  impaired  ;  syllables  are 
€lided;  there  is  a  blurred,  somewhat  explosive  utterance,  and  there  may 
be  with  this  an  occasional  hesitation.  There  is  no  twitching  of  lips, 
but  occasional  jerky  movements  have  been  noted  in  the  tongue.  The 
affection  of  speech  is  not  often  an  early  symptom.  It  may  not  be 
noticed  until  three,  five,  or  ten  years  after  the  onset  of  the  other 
symptoms.  In  most  cases  (but  not  in  all)  there  is  nystagmus  when 
the  eyes  are  moved  laterally  or  upwards.  Sometimes  the  movement  is 
slower  than  in  most  forms  of  nystagmus,  and  it  is  rarely  present  when 
the  eyes  are  at  rest,  directed  straight  forwards.  This  symptom  may 
come  on  after  those  in  the  hmbs,  but  careful  examination  will  often 
reveal  it  in  the  early  stage.  Paralysis  of  the  ocular  muscles  is  very 
rare  ;  in  one  case  there  was  strabismus  with  double  vision,*  and  slight 
diplopia  sometimes  exists  for  a  time  and  passes  away.  Optic  nerve 
atrophy  never  occurs.  The  pupils  are  usiially  normal,  but  several  cases 
have  been  recorded  in  which  there  has  been  loss  of  the  light  reflex. 
Whenever  this  condition  exists  evidence  of  hereditary  syphilis  should 
be  most  carefully  sought  for,  as  it  is  probable  that  many  of  the  cases  in 
which  this  loss  was  met  with  were  cases  of  true  tabes,  with  some 
involvement  of  the  lateral  columns  occurring  in  young  persons,  and 
should  not  be  classed  with  the  disease  in  question. 

Sensory  symptoms  are  very  variable.  Lightning  pairs  and  any 
severe  pains  are  extremely  rare,  though  in  one  or  two  recorded  in- 
stances they  have  been  severe  ;  but  slight  dull  or  rheumatoid  pains  in 
the  legs  are  not  uncommon.  Sensibility  has  been  quite  normal  in 
many  cases  even  of  severe  degree;  in  others  there  has  been  slight 
anaesthesia  in  the  legs,  early  in  some  instances,  late  in  others.  In  one 
case  there  was  ■  delay  of  conduction,  such  as  is  met  with  in  tabes  ;  f 
rarely  sensibility  to  pain  and  temperature  is  impaired.  The  sense  of 
posture  of  the  limbs  has  been  found  normal  in  several  cases  in  which 
•  Charcot,  'Prog.  Med.,'  1887,  No.  23. 


522 


SriNAL    CORD. 


it  lias  been  examined.  The  electro-sensibility  of  tbe  muscles  is  said 
to  be  sometimes  lessened.  Increased  sensitiveness  to  pain  is  occa- 
sionally met  witli.  Reflex  action  from  tbe  sole  is  "usually  preserved, 
but  may  be  lost  wben  there  is  ansesthesia.  The  cremaster  reflex  ia 
often  lost.  On  account  of  the  age  of  the  patients  little  is  known  of 
the  condition  of  sexual  power,  but  it  is  certainly  often  absent. 
Menstruation  xisually  becomes  irregular  and  ceases.  The  sphincters, 
as  a  rule,  are  unaffected.  There  is  no  tendency  to  trophic  changes  in 
the  skin  or  joints. 

Although  muscular  power  is  commonly  normal  at  first,  and  may  be 
normal  even  when  the  ataxy  is  considerable,  it  usually  becomes  impaired 
as  the  disease  progresses,  and  sometimes  weakness  comes  on,  with  the 
ataxy,  at  the  onset  of  the  disease.  The  loss  of  power  is  alwaya 
greatest  in  the  legs,  and  may  be  confined  to  them.     The  flexors  suffer 


Yl&.   138. — Showing  a  condition  of  marked  lateral  curvature  in 
Friedreich's  ataxy. 


more  than  the  extensors,  and  the  weakness  in  the  flexors  of  the  ankle 
may  permit  some  degree  of  talipes  to  occur.  The  loss  of  power  may 
be  ultimately  great,  although  it  rarely  amounts  to  absolute  paralysis. 
Slight  wasting  of  the  muscles  may  occur  in  the  later  stages  of  the 


HEREDITAEY   ATAXY 


523 


disease,  attended  witli  onlv  trifling  depression  of  tlie  electrical  con- 
tractility ;  in  two  recorded  eases,  however,  a  brother  and  sister,  there 
was  great  atrophy  of  mnscles  with  altered  electrical  reactions.* 
Tiateral  curvature  of  the  spine,  and  talipes  equinus  or  equino-varns 
of  the  feet  (see  Figs.  138  and  139.),  are  common  as  later  results  of  the 


Fia.  130. — Showing  the  characteristic  deformity  of  the  feet  iu 
Friedreich's  ataxy. 

muscular  weakness,  developing  under  I  he  influence  of  posture;  and 
contraction  of  the  flexors  of  the  knees  has  also  resulted. 

Visceral  crises  do  not  occur.  Frequency  of  pulse  has  been  noted, 
however,  in  many  cases,  and  curious  vaso-motor  symptoms  (flushing, 
oedema,  sweating,  polyuria,  and  salivation)  were  present  in  one  of 
Friedreich's  cases.  A  basic  cardiac  murmur  is  of  very  freqvient 
occurrence,  possibly  associated  with  the  anaemia  which  is  frequently 
present.  There  is  no  mental  change  that  can  be  regarded  as  part  of 
the  disease,  although  imbecility  has  co-existed  (Power)  .f 

The  isolated  cases  may  correspond  closely  to  the  type,  presenting 
the  same  nystagmus,  weakness,  and  unsteadiness ;  many  of  these 
cases,  however,  differ  somewhat,  although  sufficiently  characteristic  in 

*  Dejerjne,  'Med.  mod.,'  1890,  No.  2.5. 

t  An  excellent  analysis  of  the  symptoms,  &c.,  is  given  by  Crozier GriflSth  (*  Trans. 
Coll.  Pliys.  Philaddphia,'  1888;  and  by  Ladame  ('Brain/  1890,  pt.  52),  and 
Mackie  Whyte  ('  Brain,'  1898). 


524  SPINAL    COED. 

tlieir  main  features  to  justify  their  inclusion  Speecli  more  often 
escapes.  I  iiave  once  met  with  a  congenital  lisp.  In  some  the  sym- 
ptoms are  slight,  and  have  developed  late.  A  difficulty  in  micturition 
may  precede  other  symptoms.  There  are  also  cases,  sporadic  and 
grouped,  in  which  coarse  tremor  is  conspicuous,  and  the  condition 
might  be  regarded  as  one  of  simple  tremor,  were  it  not  for  the  presence 
or  development  of  other  symptoms.  For  instance,  one  man  had 
clipping  speech  "all  his  life;"  between  forty  and  fifty  difficulty  in 
writing  came  on,  owing  to  a  peculiar  spasmodic  tremor  in  both  arms, 
brought  on  also  in  the  legs  by  exertion;  at  fifty  unsteadiness  on 
walking  was  added.  There  was  a  history  of  a  similar  affection  in 
several  relatives. 

The  rate  of  progress  of  the  malady  varies  much,  even  in  different 
members  of  the  same  family.  One,  in  whom  the  disease  commenced 
last,  may  be  unable  to  walk,  while  another  who  suffered  sooner  may 
be  stiU  in  the  early  stage  of  the  disease.  Now  and  then  the  disease  is 
stationary  for  many  years.  The  duration  of  the  malady  is  corre- 
spondingly variable.  It  is  always  long,  even  more  than  thirty  years, 
and  in  many  instances  it  has  not  apparently  shortened  life.  On 
the  other  hand,  death  may  occur  at  the  end  of  ten  or  twelve  years. 
The  end  has  generally  come  from  intercurrent  affections,  rarely  from 
exhaustion.     Acute  myelitis  has  also  caused  death  (Everett  Smith). 

A  few  years  ago,  from  a  consideration  of  the  results  of  his  own 
observations  and  those  of  others,  Marie*  concluded  that  there  exists  a 
type  of  disease  in  many  points  resembling  Friedreich's  ataxy,  but 
differing  in  several  important  particulars  This  he  called  hereditary 
cerebellar  ataxy,  and  he  regarded  the  symptoms  as  the  result  of  some 
congenital  defect  in  the  cerebellum.  In  the  ataxy,  the  articulatory 
difficulty,  the  frequent  presence  of  nystagmus,  and  the  fact  that  the 
disease  might  occur  in  more  than  one  member  of  the  same  family,  the 
condition  closely  resembled  Friedreich's  disease,  but  striking  differences 
were  manifested  in  the  occasional  presence  of  the  Argyll  Eobertson 
pupil,  the  not  unusual  occurrence  of  optic  atrophy,  and  the  invariable 
exaggeration  of  the  knee-jerk.  The  onset  of  symptoms  also  was 
usually  much  later  in  this  disease.  The  remarkable  series  of  cases 
described  by  Dr.  Sanger  Brownf  probably  belong  to  this  class.  They 
are  characterised  by  the  occurrence  of  a  similar  condition  in  many 
members  of  the  same  family  throughout  several  generations,  viz. 
gradually  increasing  weakness  and  inco-ordination  in  the  legs,  with 
marked  tremors  in  the  head  and  body,  impaired  articulation,  exag- 
gerated knee- jerk,  occasional  ankle-clonus,  and  frequently  optic  atrophy. 
There  was  no  nystagmus  and  no  deformity.  Nonne  and  Menzel  have 
also  described  similar  conditions,  but  in  Nonne's  cases  the  ocular 
movements  were  impaired,  and  there  was  mental  weakness.     G-ee.J 

*  '  La  Semaine  Medicale,'  1893. 

t  'Brain,'  1892,  Summer. 

J  '  St   Brtrt.'s  Hosp.  E,ep.,'  voL  xxv. 


HEREDITAET   ATAXY. 


525 


Tooth,*  and  others  have  also  described  a  family  disease  characterised 

by.  symptoms  of  spastic  paraplegia  (lateral  sclerosis) . 

Pathological  Anatomy.— The  lesion  in  hereditary  ataxy  is  that 
of  ataxic  paraplegia  and  of  tabes  combined.  There  is 
degeneration  in  the  lateral  column,  often  also  in  the 
anterior  column,  such  as  occurs  in  ataxic  paraplegia , 
but  the  sclerosis  of  the  posterior  column  is  more 
intense  than  in  that  disease,  and  it  is  also  more  ex- 
tensive, especially  in  tl.e  lumbar  region  It  is  simi- 
lar to  the  degeneration  of  tabes,  and  agrees  with  this 
also  in  that  the  posterior  nerve-roots  are  usually 
affected,  whereas  in  ataxic  paraplegia  they  almost 
invariably  escape. 

The  distribution  of  the  lesions  shows  a  close  cor- 
respondence in  different  cases,  as  will  be  seen  by  a 
comj)arison  of  the  figures  here  given,  which  are  from 
three  different  cases  of  the  disease.      The  sclerosis 


Fl&.  140. — Hereditary  ataxy ;  •section  of  spinal  cord 
at  first  lumbar  segment.  Sclerosis  of  the  whole  of 
the  posterior  columns  except  a  narrow  zone  adja- 
cent to  the  neck  of  the  horn.  Degeneration  also 
of  the  lateral  pyramidal  tracts,  and,  in  front  of 
this,  sliglit  degeneration  in  the  superficial  layer  of 
the  lateral  column.  Adjacent  to  the  anterior 
median  fissure  there  is  also  a  zone  of  sclerosis  of 
the  anterior  pyramidal  tract,  which  extends,  in 
this  cord,  into  the  lumbar  region.f 


FlO.  141.— Heredi- 
tary ataxy;  dis- 
tribution of  de- 
generation in  the 
white  columns, 
indicated  by  the 
dotted  shading. 
(After  Fried- 
reich.) 


•  •  St.  Bart.'s  Hosp.  Eep.,'  vol.  xxvii. 

t  I  am  indebted  for  this  section  to  Dr.  Everett  Smith,  who  hag  published  the  case 
in  the  '  Boston  Med.  and  Surg.  Journal,'  Oct.  15th,  1885.  The  patient  was  one  of  three 
sisters  aff'ected  with  the  disease,  the  brothers  being  healthy.  The  father  sufl'ered 
from  ataxic  paraplegia  late  in  life.  In  the  case  from  which  the  drawing  is  made 
the  inco-ordi nation  began  in  the  legs  at  the  age  of  nineteen,  and  in  the  arms  goon 
afterwards,  and  was  quickly  followed  by  loss  of  power,  which  gradually  increased 
to  almost  universal  paralysis,  with  muscular  contractures  (talipes  equino-varus,  &c.) 
and  considerable  loss  of  sensation.  There  were  nystagmus,  afifection  of  articulation, 
and  some  mental  dulness.     Death  occurred  at  the  age  of  forty. 


626 


SPINAL   CORD. 


t'la.  142. — Hereditai^  at.ixy.  A,  cervical;  B, 
dorsal  j  C,  lumbar  regions  of  the  cord.  The 
posterior  columns  are  sclerosed  in  their  whole 
extent,  except  in  the  vicinity  of  the  neck  of 
the  horn  in  the  cervical  and  lumbar  regions, 
the  degeneration  being  rather  less  intense  in 
the  lumbar  than  in  the  other  parts.  In  the 
antero-lateial  columns  there  is  an  annular  zone 
of  sclerosis  in  the  periphery  of  the  cord,  widen- 
ing in  the  region  of  the  pyramidal  traqt.  In 
front  of  this  tract  tlie  zone  is  widest  in  the 
dorsal  region,  and  trifling  in  degree  in  the 
lumbar.  The  grey  matter  is  but  little 
affected.* 


of  the  posterior  coltunna 
may  be  complete  through- 
out the  cord,  with  the  ex- 
ception of  a  narrow  band 
near  tlie  neck  of  the  cornu, 
which  always  remains  but 
little  damao-ed.  In  the 
lumbar  region  it  is  usually 
intense  up  to  the  head  of 
the  horn  and  the  posterioi 
surface  of  the  cord  ;  occa- 
sionally, however,  it  is  some- 
what less  intense  in  this  re- 
gion than  it  is  higher  up, 
extending,  nevertheless,  into 
the  root-zone.  In  the  cer- 
vical region  the  sclerosis 
may  also  be  general,  or  it 
may  be  greatest  in  the  root- 
zone  and  in  the  posterior 
median  columns.  The  cer- 
vical root-zone  never  ■  es- 
capes, as  it  often  does  in 
tabes. 

The  degeneration  of  the 
lateral  columns  always  in- 
volves the  pyramidal  tracts, 
and  is  most  intense  in  their 
position.  It  is  not,  how- 
ever, Umited  to  them.  It 
usually  extends  outwards  to 
the  periphery  of  the  cord, 
even  where  the  pyramidal 
tract  does  not  reach  the 
surface,  thus  involving  the 
direct  cerebellar  tract ;  and 
it  also  extends  forwards  at 
the  surface,  so  as  to  con- 
stitute a  band  of  annular 
sclerosis,  which  varies  ia 
thickness  in  different  parts 
of  the  cord  and  in  different 
cases  (rigs.  141,  142).     In 


*  For  the  sections  from  which  the  drawing  is  made  I  am  indebted  to  Dr.  Pitt 
(who  prepared  them)  and  the  late  Dr.  Moxon,  under  whose  care  the  patient  died  in 
Guy's  Hospital.  The  case  (full  details  of  which  will  be  found  in  a  recent  volume  of 
the  *  Guy's  Hospital  Reports  ')  is  one  of  a  series  of  five  cases  (four  brotherg  and  « 


HEEEDITARY    ATAXY. 


527 


the  inner  part  of  the  anterior  column  there  may  be  a  distinct  area 
of  degeneration  in  the  position  of  the  anterior  pyramidal  tract.  This 
is  seen  in  Fig.  140,  in  which  this  tract  extends  unusually  low  in  the 
cord,  and  is  distinctly 
degenerated  m  the 
upper  part  of  the 
lumbar  enlargement. 
Atrophy  of  the  poste- 
rior vesicular  column 
has  been  found  a.  :o-  f 
ciated  with  that  of  the 
direct  cerebellar  tract. 
Slight  abnormal  ap- 
pearances  have  been 
seen  in  the  nerve- cells 
of  the  anterior  cornua 
in  some  cases,  but  as  a 
rule  these  are  normal, 
and  the  grey  matter 
presents  no  other  dis- 
ease. It  is  very  likely, 
however,  that  future 
observations  will  reveal 
the  occurrence  of 
changes  in  the  nerve- 
cells  of  the  posterior 
horns,  such  as  are  met 
with  in  tabes. 

The  pia  mater  over  the  posterior  columns  has  been  found  thickened. 
The  posterior  nerve-roots  usually  present  some  degeneration  of  their 
fibres;  this  may  be  partial  (Fig.  143,  b),  or  may  amount  to  total 
destruction  of  the  fibres  (as  at  c).  The  peripheral  nerves  in  the 
limbs  have  been  found  normal.* 

Some  general  shrinking  and  induration  of  the  pons  and  medulla 
were  found  by  Friedreich,  with  atrophy  of  the  cells  of  the  post- 
pyramidal  nucleus  and  some  degeneration  of  the  restiform  bodies,  but 
none  of  the  anterior  pyramids.  Corpora  amylacea  were  present  in 
the  hypoglossal  nerves. 

Atrophy  of  the  cerebellum,  pons,  and  olivary  bodies  was  found  in  one 
case  ;  the  cells  of  Purkinje  were  diminished  in  numbers,  but  showed 
no  sign  of  atrojohy  or  degeneration. f  There  was  also  degeneration  of 
the  hypoglossal  and  facial  nuclei. 

This  defective  cerebellar  development  is  the  condition  supposed  to 

sister)  which  I  reported  some  years  a,;o  to  the  Clinical  Society  ('  Clia.  Soc  Trans./ 
vol.  xiv,  1881,  p.  1). 

*  Friedreich,  •  Virchow's  Archiv,'  Bd.  Ixx,  p.  145. 

t  P.  Meuzel,  'Arch.  f.  Psych./  Bd.  xxii,  H.  1,  p.  160. 


VC 


Fia.  143. — Hereditary  ataxy  J  degeneration  of  poste- 
rior nerve-roots  (from  the  same  case  as  Fig.  142).  A, 
normal  interior  root,  for  comparison.  B,  posterior 
root,  partially  desenerated ;  a  few  normal  nerve- 
fibres  are  seen,  but  most  of  the  spaces  which  should 
contain  nerve-filires  are  empty.  C,  posterior  root, 
totally  degenerated,  probably  by  a  more  acute  pro- 
cess than  that  of  B,  since  there  is  some  destruction 
of  the  septa  and  increase  of  amorphous  connective 
tissue.  The  products  of  degeneration  are  rendered 
invisible  by  the  mode  of  preparation.  (Compare  C 
with  the  similar  chano^es  in  a  totally  degenerated 
peripheral  nerve  shown  in  Pig.  59  B,  p.  165.) 


528  SPINAL   COED. 

underlie  tlie  manifestations  of  tlie  condition  already  alluded  to  as  the 
hereditary  cerebellar  ataxy  of  Marie.  In  another  case  *  the  anatomical 
condition  reyealed  the  absence  of  cord  degeneration,  but  the  cere- 
bellum was  small,  and  the  cells  of  Purkinje  deficient.  In  the  only 
one  of  Sanger  Brown's  examined,  however,  there  were  sclerotic  changes 
in  the  lateral  and  posterior  columns,  but  no  marked  cerebellar  changes 
were  present,  although  the  cells  of  Purkinje  are  said  not  to  have  been 
over-abundant.  A  case,  similar  in  clinical  conditions,  was  many  years 
ago  described  by  Eraser  and  Coats  of  Glasgow,  and  in  this  also  there 
was  no  cord  change,  but  marked  atrophy  of  the  cerebellum,  and  a  de- 
fective development  of  the  cells  of  Purkinje.  It  is  of  some  interest 
that  a  condition  has  been  described  in  cats  congenitally  ataxic,  in 
which  the  cerebellum  is  small,  and  Purkinje's  cells  absent,  or  defective 
in  character  and  number. 

Pathology. — The  disease  apparently  occupies  a  clinical  and  patho- 
logical position  between  the  combined  sclerosis  described  as  ataxic 
paraplegia  and  s^imple  tabes,  resembling  the  former  in  the  common 
affection  of  the  lateral  and  posterior  columns,  and  the  consequent 
weakness  with  ataxy — resembling  it  aTso  in  the  imperfect  hmitation  of 
the  changes,  and  in  the  intensity  of  those  in  the  middle  of  the  pos- 
terior columns  in  the  dorsal  region ;  but  it  differs  from  this,  and 
resembles  tabes,  in  the  degree  of  affection  of  the  posterior  columns 
in  the  lumbar  regions,  the  extension  in  sHght  degree  into  the  root- 
zones,  and  the  affection  of  the  posterior  roots  :  with  these  the  loss  of 
the  knee-jerk  and  the  affection  of  sensation  are  apparently  connected. 
The  isolated  cases,  occasionally  met  with,  present  the  same  combina- 
tion of  spinal  symptoms.  The  precise  origin  of  the  affection  of 
articulation  and  of  the  nystagmus  has  not  yet  been  traced. 

The  age  at  which  the  disease  commences,  long  before  the  period 
at  which  ordinary  degenerations  occur,  and  its  family  grouping, 
suggest  that  its  ultimate  cause  is  a  congenital  tendency  of  develop- 
ment, by  which  the  affected  elements  have  a  briefer  period  of  vital 
endurance  than  the  other  tissues  of  the  organism.  Mobius  has 
suggested  that  there  is  an  actual  arrest  of  develojoment  of  these 
structures,  but  it  is  evident  that  their  development  suffices  for  perfect 
function  during  the  early  part  of  life,  and  that  their  functional 
capacity  undergoes  subsequent  failure,  which  can  only  be  due  to  a 
process  of  structural  change.  It  is  possible  that  we  have  here,  again, 
a  double  morbid  process,  and  that  a  tendency  to  early  degeneration  in 
the  nerve-elements  is  associated  with  a  converse  tendency  to  over- 
growth of  the  interstitial  tissue,  analogous  to  that  which  we  have  in 
the  muscles  in  pseudo-hypertrophic  paralysis  (q.  v.).  In  this  con- 
nection it  is  important  to  note  that  in  one  case  the  neuroglial  over- 
growth suggested  a  pure  increase  of  the  normal  tissue,  rather  than  a 
result  of  inflammation ;  and  the  peripheral  sensory  nerves,  although 

•  '  Notme, '  Arch,  f .  Psych.,'  xxii,  1891. 


"  HEREDITARY  ATAXY."  529 

not  degenerated,  contained  a  considerable  nnmber  of  embryonal  nerve- 
fibres  hi  the  fasciculi.*  Dejerine  would  regard  the  neuroglial  increase 
as  the  sole  element  in  tbe  morbid  process ;  but  the  fact  last  mentioned, 
as  Tvell  as  the  preponderant  affection  of  certain  tracts,  makes  it 
improbable  that  this  exclusive  view  is  correct. 

Although  we  have  here  only  to  consider  hereditary  cerebellar  ataxy 
and  similar  conditions,  in  relation  to  Friedreich's  ataxy,  it  may  be 
stated  that  in  this  disease  also  we  probably  have  to  deal  with  a 
developmental  defect  or  perversion,  as  has  been  indicated  in  con- 
sidering the  morbid  anatomy.    And  the  same  may  be  said  of  the 
family  form  of  spastic  paralysis.     It  might  already  seen  as  if  we  had 
a  series  to  deal  with,  with  Friedreich's  ataxy  at  one  end  and  family 
spastic  paralysis  at  the  other,  the  intermediate  space  being  occupied  by 
cases  like  those  of  Sanger  Brown,  in  which  spasticity  and  ataxy  are 
combined,  and  in  which  the  underlying  anatomical  condition  as  regards 
the  cord  is  one  of  sclerosis  affecting  lateral  and  posterior  tracts  fairly 
equally,  while  in  Friedreich's  disease  we  have  to  deal  with  a  condition 
in  which  the  posterior  columns  suffer  most,  and  in  family  spastic  para- 
lysis with  one  in  which  the  lateral  columns  may  be  exclusively  affected. 
Diagnosis. — In  most  cases  the  inco-ordination  is  sufficiently  pre- 
dominant to  suggest  that  the  disease  is  a  form  of  ataxy,  and  the 
unsteadiness  of  the  head,  the  affection  of  articulation,  the  nystagmus, 
and  the  age  at  onset,  suffice  for  the  distinction  from  ordinary  tabes. 
The  common  form  of  ataxic  paraplegia  bears  a  close  resemblance  to 
this  disease,  a  resemblance  that  is  more  than  superficial,  although  the 
excessive  knee-jerk  and  foot-clonus,  almost  always  present  in  ataxic 
paraplegia,  are  usually  absent  in  hereditary  ataxy ;  much  spasm  is 
always  absent,  and  nystagmus  is  common.    The  difference  in  the  state 
of  myotatic  irritability  is,  as  we  have  seen,  not  absolute;    cases  of 
combined  lateral  and  posterior  sclerosis  occur,   of  tabetic  type,  in 
which  the  knee-jerk  is  lost,  and  in  at  least  one  case  of  hereditary 
ataxy  the  knee-jerk  has  been  excessive.    The  age  of  onset  and  family 
multiplicity  may  decide  the  question,  but  the  isolated  cases,  which 
are  by  no  means  rare,  commencing  soon  after  puberty,  are  not  dis- 
tinguishable from  the  hereditary  disease ;  they  seem  to  be  analogous 
to  the  isolated  cases  of  pseudo-hypertrophic  paralysis.    A  greater  diffi- 
culty is  presented  by  cases  of  slight  tabes  in  children,  the  subjects  of 
inherited  syphilis,  in  whom  it  is  not  uncommon  for  some  weakness  of 
the  legs  to  co-exist,  or  speech  to  be  disordered,  in  consequence  of  some 
early  lesion  of  the  brain.     The  loss  of  the  iris-reflex  should  suggest 
inherited  syphilis,  and  other  indications  of  this  will  often  decide  the 
nature  of  the  case. 

Disseminated   sclerosis  presents  inco-ordination,  nystagmus,  and 

impaired  articulation,  but  the  ataxy  of  the  arms  differs  in  the  wide 

range  and  violent  character  of  the  disordering  jerks  which  characterise 

this  disease ;  while  simple  unsteadiness  of  the  legs  is  very  rare,  and 

*  Auscher, '  La  Semaine  Med.,'  1890,  No.  32. 

VOL.  I.  34 


530  SPINAL    COED. 

the  course  of  tlie  malady  is  more  rapid.  The  affection  of  speech 
differs  from  that  of  hereditary  ataxy  in  being  simply  "  staccato,"  with 
undue  separation  of  syllables,  in  the  marked  cases.  In  many  in- 
stances, however,  the  articulatory  defect  is  similar  in  the  two  dis- 
eases.    The  cases  are  always  isolated. 

Cerebellar  tumour  and  hereditary  ataxy  can  hardly  be  confounded, 
in  spite  of  the  fact  that  the  unsteadiness  in  walking  is  very  similar  in 
the  two  diseases ;  the  common  affection  of  the  arms  in  the  one,  and 
the  conspicuous  head  symptoms  (severe  pain,  optic  neuritis,  &c.)  of 
the  other,  sufficiently  distiaguish  them.  It  is  very  common  for  the 
tremor  of  the  head  to  be  at  first  ascribed  to  mere  "  nervousness,"  and 
also  for  the  isolated  form,  in  girls,  to  be  regarded  as  hysterical.  The 
presence  of  nystagmus  should  at  once  decide  the  question;  it  is 
absolute  proof  of  definite  disease. 

Peognosis. — The  prognosis  in  every  case  is  very  serious,  since  the 
disease,  being  a  developmental  affection,  is  essentially  progressive;  but 
life  may  be  prolonged  for  many  years,  and  in  slight  cases  the  malady 
may  interfere  comparatively  little  with  the  patient's  occupation.  Thus 
I  have  known  a  man,  with  very  marked  symptoms,  follow  his  business 
as  a  tradesman  for  many  years.  The  only  guide  to  individual  prognosis 
is  the  observed  rate  of  progress,  which  has  little  relation  to  the  age  at 
which  the  symptoms  commence. 

Treatment. — As  in  other  diseases  that  depend  on  a  congenital 
tendency,  treatment  is  almost  powerless.  The  measures  recommended 
for  ordinary  locomotor  ataxy  (apart  from  those  suggested  by  the 
special  symptoms  of  tabes)  are  those  most  suitable  to  the  hereditary 
form.  Arsenic,  phosphorus,  and  nitrate  of  silver  deserve  a  trial ;  now 
and  then  they  seem  to  check  the  progress  of  the  disease  for  a  longer 
or  shorter  time,  but  its  individual  tendency  determines  its  course, 
almost  irrespective  of  treatment. 


SIMPLE    SENILE    PARAPLEQIA. 

This  name  seems  the  most  unobjectionable  for  a  condition,  some- 
what rare,  which  appears  hitherto  to  have  been  unrecognised.*  It  is 
confijied  to  late  life,  occurring  in  those  over  40,  and  especially  over  50. 
Its  characteristic  is  simple  weakness  of  the  legs,  with  some  slowness  of 
movement,  but  without  wasting,  sensory  disturbance,  or  reflex  altera- 
tion. The  knee-jerk  is  normal,  and  there  is  no  foot-clonus.  The 
malady  develops  very  gradually  and  is  slowly  progressive,  although  it 
seems  seldom  to  attain  such  a  degree  as  to  prevent  standing.  The 
<jondition  of  the  legs  resembles  that  in  cases  of  paralysis  agitans  with- 
out tremor,  in  which  the  malady  is  manifested  only  by  weakness,  and 
*  A  note  upon  it  by  myself  appeared  in  the  'Ceutralbl.  f.  Nervenkr.,'  1890. 


OHEONIO    MUSCULAE  ATROPHY.  531 

stiff  slowness  of  movement  of  the  limbs,  face,  and  trunk.  Cases  are 
met  with  in  which  the  condition  of  the  legs  above  described  is  asso- 
ciated with  slight  symptoms  in  the  arms  and  face,  such  as  characterise 
these  cases  of  paralysis  agitans.  Thus  senile  paraplegia  is  probably  a 
partial  development  of  the  morbid  process  of  that  disease ;  instead  of 
being  general,  it  is  limited  to  the  structures  for  the  legs.  If,  as  the 
condition  seems  to  suggest,  paralysis  agitans  depends  on  peculiar 
changes  in  the  nutrition  and  function  of  the  motor  cells  of  the  cortex 
cerebri  (which  impair  the  power  and  alter  the  action  of  the  cells,  but 
do  not  usually  go  on  to  such  destructive  alteration  as  entails  secondary 
degeneration  of  the  pyramidal  fibres),  we  can  readily  conceive  that 
such  a  process  may  be  sometimes  limited  to  the  leg-centres,  and  may 
cause  the  symptoms.  If  so,  the  malady  is  a  cerebral  and  not  a  spinal 
one,  but  it  is  mentioned  here  on  account  of  its  paraplegic  features, 
and  their  resemblance  to  those  of  disease  of  the  spinal  cord.  Degene- 
rative changes  may,  however,  be  various  in  kind  and  degree,  especially 
in  late  life ;  cases  occur,  similar  to  these,  but  with  myotatic  excess  con- 
necting them  with  the  destructive  changes  of  lateral  sclerosis,  while 
others  present  such  symptoms  as  nystagmus  or  impeded  articulation — 
evidence  that  the  altered  nutrition  is  wider  in  its  range. 


CHRONIC    SPINAL   MUSCULAR  ATROPHY. 

(PBOGEESSIVB    MT7SCULAE    ATEOPHT ;    WASTING    PAIiST;    AMTOTEOPHIO 
LATEBAL   SCLEEOSIS ;    CHEONIC   POLIO-MYELITIS). 

The  disease  which  has  long  been  known  by  the  names  "  progressive 
muscular  atrophy"  and  "wasting  palsy"  is  characterised  by  slow 
wasting  of  the  muscles,  beginning  in  some  one  part,  and  usually 
spreading  and  increasing  until  it  is  wide  in  extent  and  extreme  in 
degree.  The  changes  in  the  muscles  depend  on  changes  in  the  spinal 
cord,  a  slow  degeneration  of  the  ganglion-cells  of  the  anterior  cornua, 
accompanied  by  a  similar  degeneration  in  the  motor  nerve-fibres  which 
spring  from  the  cells.  With  this  degeneration  of  the  cells  and  peri- 
pheral fibres  there  is  usually  also  associated  a  degeneration  of  the 
pyi-amidal  tracts  in  the  cord,  sometimes,  at  least,  to  be  traced  up  to 
the  motor  cortex  of  the  brain.  According  to  cun-ent  views,  there  is  a 
decay  of  the  spinal  motor  neuron,  and  often  also  of  the  upper  neuron, 
which  has  its  nutritional  centre  in  the  cells  of  the  motor  cortex. 

Chronic  muscular  atrophy  was  separated  from  primary  paralysis  by 
Sir  Charles  Bell,  and  was  afterwards  described  more  fully  by  Aran* 

*  Aran,  "EechercheB,  &c.,  sur  Faraljsie  Mascalaire  Atropbiqae/' '  Aieh.  g6n.  da 
Med.;  1850. 


532  SPINAL  CORD. 

and  Cruveilhier.*  Bell  and  Cruveilhier  regarded  it  as  a  spinal  diseasev 
Aran  (who  first  called  it  "  progressive  muscular  atrophy ")  thouglit 
that  it  was  primarily  a  disease  of  the  muscles,  a  view  that  was  after- 
wards held  by  Duchenue,t  and  subsequently  also  by  Friedreich.  This 
opiaion  was  supplemented  by  a  theory  that  the  wasting  was  due  to  a 
disturbance  of  the  sympathetic.  Luys  |  first  discovered  the  disease  of 
the  grey  substance  of  the  spinal  cord,  and  Charcot  especially  associated 
the  atrophy  with  the  wasting  of  the  ganglion- cells.  When  the  con- 
stancy of  the  spinal  lesion  was  demonstrated,  it  was  thought  that  every 
form  of  chronic  muscular  atrophy  was  of  spinal  origin ;  but  this  view 
has  proved  erroneous,  and  it  has  been  shown  that  there  are  forms  of 
muscular  atrophy  which  are  purely  local  and  idiopathic.  The  morbid 
process  is  not  confined  to  the  spinal  cord.  Atrophy  of  the  nerve-cells 
from  which  the  motor  bulbar  nerves  arise  often  accompanies  that  of 
the  spinal  cells,  and  "  progressive  bulbar  paralysis  "  is  then  associated 
with  "  progressive  muscular  atrophy :  "  it  often  comes  on  after  the  dis- 
ease has  existed  for  some  time,  and  ends  life ;  occasionally  it  initiates 
the  malady. 

Two  varieties  of  the  disease  have  been  distinguished  by  Charcot, 
who  has  been  followed  in  this  by  most  subsequent  writers.  In  the 
one  variety  the  disease  is  manifested  only  by  the  wasting  of  the  mus- 
cles— the  " Aran-Duchenne  type;"  in  the  other  there  are,  in  parts 
other  than  those  much  atrophied,  the  indications  of  degeneration  of 
the  pyramidal  tracts  of  the  cord.  In  the  latter  cases,  Charcot  re- 
garded the  degeneration  of  the  pyramidal  tracts  as  the  primary 
lesion,  and  the  affection  of  the  cornua  as  secondary.  He  therefore 
termed  the  cases  of  the  first  class  "  protopathic,"  and  those  of  the 
second  class  "  deuteropathic,"  and  gave  to  the  latter  the  name  "  amyo- 
trophic lateral  sclerosis."  In  G-ermany,  however,  and  especially  by 
Leyden,  doubts  have  been  expressed  as  to  the  vahdity  of  the  dis- 
tinction between  the  two  classes  of  cases,  and  as  to  the  sequence  of 
the  lesions  in  the  second  class.  These  doubts  are  not  without  founda- 
tion. The  subject  will  be  discussed  when  the  pathology  of  the  disease 
is  considered,  and  reasons  will  then  be  given  for  the  course  here 
adopted  of  considering  the  two  varieties  together  as  essentially  one 
disease. 

But  cases  are  also  met  with  (although  not  frequently)  that  present 

•  Cruveilhier,  'Arch.  gen.  de  Medecine,'  1853.  Hence  the  disease  has  been  often 
termed  "  Cruveilhier's  atrophy." 

t  Duehenne, '  Bull.  gen.  de  Therap.,'  1§53. 

X  In  1860.  Lockliart  Clarke  described  in  1861  changes  (lacunse)  in  the  spinal 
cord,  but  in  the  anterior  horn  cells  only  pigmentation,  not  atrophy.  Other  details 
of  the  history  of  the  distinction  of  the  disease  and  the  discovery  of  its  nature  will 
be  found  in  *  A  History  of  the  Chronic  Degenerative  Diseases  of  the  Central  Nervous 
System,*  by  T.  K.  Monro,  M.A.,  M.D.,  Glasgow,  1895,  an  admirable  account  of  the 
stages  of  the  discernment  of  all  these  diseases.  To  this  book  the  reader  may  he 
referred  for  more  particulars  regarding  the  subject  than  can  be  given  here,  not  only 
in  regard  to  this  affection  but  to  all  others  of  a  degenerative  character. 


CHRONIC    MUSCULAR   ATEOPHT.  533 

•every  gradation  to  subacute  polio-myelitis  in  one  direction,  and  per- 
haps also  to  polyneuritis  in  another.  Attempts  are  being  made  to 
classify  these,  but  types  may  be  multiplied  indefinitely  from  the  inter- 
mediate forms,  and  many  more  pathological  and  clinical  observations 
are  necessary  before  a  useful  classification  of  the  cases  can  be  made. 
In  the  following  description  the  common  forms  are  chiefly  considered. 

CA.USES.  — The  disease  is  more  frequent  in  males  than  in  females,  the 
proportion  being  about  three  to  one.  It  is  chiefly  a  disease  of  adult 
life,  commencing  usually  between  twenty-five  and  forty-five.  I  have 
known  it  to  begin  at  fourteen,  and  as  late  as  seventy,  and  a  case  is 
recorded  in  whirh  it  began  at  twelve ;  *  there  was  wasting  and  rigidity 
in  the  limbs,  tongue,  lips,  and  lower  face.  But  most  cases  of  muscu- 
lar atrophy  in  early  life  are  myopathic,  and  not  spinal.  Heredity  is  to 
be  traced  only  in  less  than  half  the  cases,  and  generally  as  an  indirect 
neuropathic  disposition.  Rarely  there  is  direct  inheritance  of  the 
disease.  Among  instances  that  I  have  met  with  are  a  lady  whose 
mother  died  from  a  similar  atrophy,  and  another  case  (from  which 
Fig.  146  is  taken)  in  which  a  brother  had  died  from  some  chronic  disease 
of  the  cord,  attended  with  wasting ;  but  three  cases  with  bulbar  sym- 
ptoms have  been  recorded,  two  of  whom  were  cousins,  the  childi'en  of  two 
listers  :  the  third,  who  had  had  syphilis,  was  a  cousin,  but  his  affection 
was  untypical.f  Direct  inheritance  seems  to  be  most  common  in  cases 
that  occur  late  in  life.  When  many  members  of  a  family  suffer  from 
muscular  atrophy,  the  malady  is  nearly  always  myopathic  and  not 
spinal.  The  affection  occurs  in  all  classes  of  society,  and  it  is  doubt- 
ful whether  workers  with  the  muscles  furnish  a  larger  proportion  of 
the  cases  than  can  be  accounted  for  by  their  greater  exposure  to  certain 
exciting  causes. 

Of  these  more  direct  causes  one  of  the  most  frequent  is  mental 
distress  and  anxiety,  and  this  is  especially  met  with  in  females  and  in 
late  life.  A  severe  fright  has  been  thought  to  excite  it.  Another 
cause  is  exposure  tc  wet  cold,  which  is  also  a  cause  of  many  other 
chronic  spinal  diseases.  Sometimes  the  exposure  has  been  habitual ; 
sometimes  a  single  exposure  has  been  effective,  and  some  neuralgic 
pains,  indicative  of  the  deleterious  influence  on  the  nervous  system, 
have  followed  the  exposure  and  connected  it  with  the  later  wasting. 
The  pain  has  been  either  in  the  part  afterwards  wasted,  or  in  some 
other,  more  common,  seat  of  neuralgia.  Thus  one  patient,  after 
remaining  in  wet  clothes  for  twelve  hours,  suffered  for  six  weeks  from 
severe  neuralgia  in  the  left  side  of  the  face,  and  then  the  muscles  of 
the  left  shoulder  began  to  waste.  Although  excessive  use  of  individual 
muscles  may  cause  them  to  waste,  it  is  doubtful  whether  this  influence 
produces  general  muscular  atrophy.  Injury  to  the  cord,  such  as  results 
from  concussion  of  the  spine,  is  a  rare  cause.  It  more  often  produces 
disseminated  myelitis,  which  may  be  manifested  by  muscular  wasting 

*  H.  Blumenthal,  "  Inaug.  Diss.,"  *  Neur.  Cent.,*  1884,  p.  376. 
t  *  Virchow's  Archiv,'  1889,  p.  116. 


534  SPINAL    CORD. 

combined  with  other  symptoms.  In  a  few  instances  I  have  known 
progressive  atrophy,  of  typical  characters,  to  slowly  follow  a  concus- 
sion, as  if  this  had  set  up  a  perversion  of  the  nutrition  of  the  nerve- 
elements.  Still  more  rarely  a  fall,  injuring  one  limb,  has  been 
followed  by  muscular  atrophy  commencing  in  this  limb  and  becoming 
general.  Thus  a  woman  fell  downstairs,  and  pitched  on  her  left  hand 
and  wrist ;  she  had  pain  in  the  arm  for  a  long  time ;  two  years  after 
the  fall  this  arm  began  to  waste,  and  the  atrophy  ultimately  became 
general.  The  relation  might  be  passed  as  an  accidental  coincidence, 
were  it  not  that  in  other  central  diseases — even,  for  instance,  in  para- 
lysis agitans — the  symptoms  may  commence  in  an  injured  limb.  In 
rare  cases,  the  disease  develops,  during  adult  life,  in  a  subject  of  old 
infantile  palsy  (polio-myeHtis),  and  may  start  from  the  most  affected 
part  (see  p.  409). 

The  disease  sometimes  succeeds  syphilis,  and  no  other  cause  may 
be  traceable.  As  with  other  degenerative  diseases,  an  interval  of 
years  elapses  between  the  primary  disease  and  its  nervous  sequel. 
The  cases  that  I  have  seen  after  syphilis  have  been  typical  in  course, 
and  evidently  degenerative  in  nature.  That  syphilis  has  some  share 
in  the  causation  of  these  cases  is  probable  from  its  relation  to  other 
diseases,  such  as  tabes  ;  but  it  is  noteworthy  that  in  one  case  the 
wasting  commenced  during,  and  in  another  directly  after,  an  energetic 
mercurial  course.  This  relation  is  intelligible  if  the  disease  is  due  to 
a  toxine,  a  result  of  syphiHs,  and  not  to  the  organisms  themselves  (see 
Tabes).  Occasionally  syphilis  and  a  neurotic  heredity  can  both  be 
traced.  In  the  cases  in  which  atrophy  has  followed  an  acute  specific 
disease,  especially  measles,  and  has  begun  in  early  life,  it  is  probable 
that  the  malady  is  a  chronic  neuritis  and  not  the  central  affection  now 
under  consideration.  Such  cases  are  known  as  of  the  "peroneal 
type  "  or  "  neuritic  form  of  muscular  atrophy,"  from  the  muscles  in 
which  wasting  in  general  is  first  obtrusive,  and  are  separately  de- 
scribed at  a  later  page.  G-eneral  muscular  atrophy  may  result  from 
lead  poisoning,  but  this  form  is  not,  as  a  rule,  progressive  in  character 
when  its  cause  has  ceased  to  act.  It  resembles  the  ordinary  form  of 
progressive  muscular  atrophy,  however,  in  seat  and  features,  and  thus 
differs  from  the  common  atrophic  palsy  of  the  extensors  that  is  pro- 
duced by  lead.  In  many  cases  of  progressive  muscular  atrophy  no  cause 
for  the  disease  can  be  traced.  When  obtrusive  symptoms  have  followed 
some  adequate  cause,  it  is  not  uncommon  for  this  to  have  been  pre- 
ceded  by  slight  symptoms,  which  show  that  the  malady  already  existed. 
A  careful  inquiry  for  such  symptoms  should  never  be  neglected. 

Symptoms. — The  definite  symptoms  of  the  disease  are  occasionally 
preceded  by  dull  aching  in  the  part  afterwards  wasted.  Sometimes 
there  is  such  pain  in  the  spine  or  elsewhere,  especially,  as  already 
stated,  in  the  cases  that  are  due  to  exposure  to  cold.  Rarely  some 
sensation  other  than  pain  precedes  the  local  symptoms.  Thus  in  one 
case  a  sense  of  coldness  j)receded  the  atrophy  in  each  limb  that  was 


CHEONIO    MUSCULAR   ATROPHY.  535 

attacked.  Weakness  and  wasting  usually  come  on  together,  but  either 
may  first  attract  the  attention  of  the  patient.  In  the  shoulder  and 
back  the  loss  of  power  is  usually  first  noticed,  and  in  such  covered 
parts  the  wasting  may  become  considerable  before  it  is  observed.  In 
the  hand  the  wasting  is  often  first  noticed,  but  sometimes  it  is  the 
impairment  of  some  delicate  action,  such  as  writing,  that  draws  atten- 
tion to  the  part.  The  affected  muscles  lose  their  proper  shape,  and 
there  is  flattening,  or  even  a  depression,  where  there  should  be  a 
prominence.  If  a  patient  is  fat,  however,  the  wasting  may  cause  at 
first  very  slight  alteration  in  the  external  part,  especially  in  the  lower 
limbs. 

The  disease  commences  in  the  arm  in  nine  tenths  of  the  cases,  and 
as  frequently  in  one  arm  as  in  the  other.  It  begins  with  almost  equal 
frequency  in  the  hand  and  in  the  shovilder  muscles.  From  the  part 
first  affected  the  disease  spreads  to  other  parts  of  the  same  limb. 
Before  it  has  attained  a  considerable  degree  in  one  limb,  it  ustially 
shows  itself  in  the  corresponding  limb  on  the  other  side ;  often  in 
the  muscles  corresponding  to  those  in  which  it  commenced,  sometimes 
to  those  affected  second  in  order  of  time.  As  the  muscles  waste 
paralysis  results  of  various  character  and  degree,  corresponding  to  the 
atrophy.  In  the  hand  the  thenar  muscles  and  interossei  are  usually 
the  first  to  suffer.  The  thenar  eminence  becomes  flattened,  and  the  base 
of  the  first  metacarpal  bone  becomes  prominent.  Of  the  interossei, 
the  atrophy  of  the  abductor  indicis  is  especially  conspicuous;  the 
normal  prominence  gives  place  to  a  hollow  beside  the  metacarpal  bone 
when  the  thumb  is  abducted.  Depressions  form  between  the  meta- 
carpal bones  on  the  back  of  the  hand,  and  also  between  the  flexor 
tendons  in  the  palm  in  consequence  of  the  wasting  of  the  lumbricales. 
The  forearm  muscles  may  be  next  involved,  the  flexors  usually  before 
the  extensors ;  and  with  the  flexors  the  supinators  may  suffer,  or  they 
may  escape  imtil  the  biceps  is  involved.  Occasionally  the  dis- 
ease begins  in  the  forearm,  and  then  especially  in  the  extensor 
muscles  of  the  fingers,  sometimes  in  those  of  the  thumb,  especially  of 
its  phalanges.  The  several  parts  of  the  long  finger  muscles  may  suffer 
unequally  ;  the  ulnar  extensors  usually  suffer  most.  Of  the  shoulder 
muscles,  the  deltoid  is  generally  the  first  to  manifest  the  disease,  and 
in  some  cases  which  begin  in  the  hand  the  deltoid  suffers  before  the 
fc>rearm  muscles.  The  rounded  contour  of  the  shoulder  becomes 
changed  (Figs.  7,  144,  145),  and  the  head  of  the  humerus  can  be 
recognised  beneath  the  acromion.  It  is  not  rare  for  part  of  the  del- 
toid to  suffer  and  part  escape :  we  have  seen  (p.  36)  that  the  several 
portions  have  different  functions  and  associations,  and  this  probably 
involves  a  relation  to  separate  groups  of  nerve-cells.  The  wasting  of 
the  deltoid  is  soon  followed  by  that  of  the  other  muscles  of  the  upper 
arm  and  of  the  scapula.  The  triceps  usually  suffers  less  and  later 
than  the  biceps,  but  sometimes  the  reverse  is  the  case.  The  supra- 
and  iafra-spinati  are  often  affected  with  the  deltoid.     In  exceptional 


536 


SPINAL    CORD. 


instances  tlie  deltoid  or  hand  muscles  escape,  and  tliere  are  casea 

(perhaps  a  special  group)   in  which,  the  upper  arm   and   shoulder 

muscles  waste  even  to  an  extreme  degree, 

and  the  forearm  and  hand  muscles  escape 

entirely. 

In  most  cases,  the  wasting  early  involves 
the  muscles  of  the  back,  and  it  sometimes 
begins  in  them.  The  middle  and  lower 
parts  of  the  trapezius  usually  suffer  first ; 
the  rhomboids  and  erectors  of  the  spine  at 
a  later  date.  The  affection  of  the  trapezius 
is  readily  recognised  if  the  patient  tries  to 
put  the  shoulder  back  CPig.  145) .  Thehighest 


Fig-.  144.  —  Progressive 
jiiuscular  atrophj. 
Wasting  of  the  raus- 
cles  of  the  back  and 
arms ;  in  the  forearm 
scarcely  any  muscular 
tissue  can  be  recog- 
nised, and  in  the  hand 
all  the  muscular  pro- 
minences have  van- 
ished. (Drawn  by  Dr. 
H.  R.  Spencer.) 


FlG.145.— Progress!  ve  musciilar  at  rophy. 
Wasting  of  right  trapezius  complete 
(the  rhomboids  remaining),  of  the  left 
trapezius  partial.  On  each  side  the 
tipper  part  of  the  trapezius  is  wasted, 
and  the  contour  of  the  neck  is  cor- 
respondingly changed.  Both  deltoids 
are  also  atrophied.  (Drawn  by  Dr. 
Spencer.) 


part  of  the  trapezius  presents  a  remarkable  indisposition  to  atrophy ; 
it  often  remains  intact  to  the  last,  and  then  may  contrast  with  the 
wasting  below  it,  standing  out  on  each  side,  like  a  cord  passing  from 
the  occiput  to  the  shoulder.  Hence  Duchenne  termed  this  part 
the  ultimum  moriens.  I  have,  however,  seen  several  otherwise  typical 
cases,  (beginning  either  in  the  deltoids  or  in  the  hands,)  in  which  the 
highest  parts  of  the  trapezii  suffered  before  the  middle  parts.  The 
levator  anguli   scapulae   also   generally  escapes,   even  when   all  the 


OHKONIO    MUSCULAE  ATROPHY. 


tmiscles  about  it  are  wasted.  Tlie  serratus,  latissimus,  and  pectoralis 
major  are  usually  affected  later.  They  may  escape  wholly  or  in  part ; 
isolated  bundles  of  the 
pectora''*s  may  waste,  the 
intervening  parts  escap- 
ing, or  either  the  upper 
or  lower  part  of  the 
muscle  may  atrophy 
alone.  According  to  the 
affection  of  the  muscles 
that  are  attached  to  the 
scapula,  the  position  of 
the  bone  changes,  and  it 
becomes  rotated  under 
the  influence  of  the 
muscles  that  are  unaf- 
fected and  unopposed 
(Fig.  7,  p.  35).  The 
muscles  that  extend  the 


Fig  146. — Progressive  muscular  atrophy.  Weak- 
ness of  the  muscles  of  the  neck.  A,  habitual 
posture  of  the  head,  inclined  backwards.  B,  posi- 
tion into  which  it  falls  if- the  patient  attempts  to 
keep  it  in  the  normal  balance.  (Drawn  by  Dr. 
Spencer.) 

head  on  the  spine  often  suffer  in  con- 
siderable degree,  and  from  this  there  results  a  difficulty  in  the 
carriage  of  the  head  (Fig.  146).  It  is  habitually  inclined  back- 
wards, so  as  to  balance  it  on  the  spine  with  but  little  muscular 
exertion ;  if  moved  forwards,  it  falls  so  that  the  chin  touches  the 
chest.  It  can  be  brought  back  into  its  former  position  only  with  diffi- 
culty ;  the  patient  has  to  incline  the  trunk  backwards,  so  as  to  bring 
the  head  nearly  into  the  vertical  position,  and  then,  with  a  sudden 
contraction  of  the  sterno-mastoids,  and  a  jerk,  the  head  goes  back  into 
its  former  posture.  The  increased  innervation  of  the  weak  extensors 
of  the  head  often  causes  a  synergic  over-action  of  the  frontalis  muscles, 
which  are  normally  associated  with  the  extensors  (so  that  the  eye- 
brows are  raised  when  the  head  is  put  back  to  look  upwards.)  The 
skin  at  the  back  of  the  neck  lies  in  transverse  folds  when  the  neck  is 
extended.  The  patient  may  be  unable,  when  lying  in  bed,  to  move  the 
headf  rom  side  to  side.  The  sterno-mastoids  also  are  often  wasted ;  either 
the  sternal  or  clavicular  part  may  be  most  affected.  In  striking  con- 
trast to  the  general  wasting  of  the  neck  is  the  condition  of  the  platysma 
myoides,  which  always  escapes,  and  may  become  hypertrophied  in  a 
vain  attempt  at  compensation. 

The  muscles  of  respiration  suffer  in  the  majority  of  cases,  and  their 
impairment  constitutes  a  grave  source  of  danger  to  life.  The  inter- 
costals  rarely  escape  altogether ;  the  diaphragm  is  involved  in  many 
cases,  and  respiration  is  then  carried  on  by  the  intercostals  and 
superior  thoracic  muscles.  On  the  other  hand,  the  intercostals  may 
suffer  much  and  the  diaphragm  remain  free;  respiration  is  then 
purely  abdominal,  and  the  walls  of  the  thorax  may  be  almost  motionless 
in  breathing,  or  there  may  be  merely  a  slight  movement  of  elevation  of 
the  upper  ribs.     The  chest  becomes  flattened  in  front,  and  narrow  from 


638  SPINAL    COED. 

before  back,  from  tlie  influence  of  atmospheric  pressure  on  the  ribs, 
unopposed  hj  the  intercostals.  Either  the  upper  or  lower  intercostals 
may  suffer  most.  When  the  diaphragm  is  paralysed  the  upper  chest 
muscles  often  suffer  more  than  the  lower,  and  a  compensatory  increased 
movement  of  the  lower  ribs  carries  forward  the  abdominal  wall,  and 
may  suggest  that  the  diaphragm  is  acting  when  it  is  not.  A  careful 
examination  will  always  prevent  the  error.  The  muscles  of  the  abdo- 
minal wall  occasionally  waste,  but  far  less  frequently  than  those  of 
the  thorax. 

"Wasting  in  the  legs  is  much  less  common  than  in  the  arms,  and  if 
it  occurs  is  usually  slighter  in  degree  ;  but  occasionally  the  disease  first 
manifests  itself  in  the  legs,  and  is  more  intense  in  them  than  elsewhere. 
The  glutei,  extensors  of  the  knee,  and  the  muscles  in  the  front  and 
on  the  outer  side  of  the  lower  leg,  are  those  that  are  most  affected. 
We  shall  presently  see,  however,  that  the  legs  are  often  paralysed 
when  they  are  not  wasted,  and  sometimes  they  are  the  seat  of  wasting 
which  differs  in  certain  features  from  that  which  is  the  especial 
characteristic  of  the  disease. 

The  face  almost  always  escapes  the  general  atrophy,  and  its  normal 
appearance  may  present  a  striking  contrast  to  the  rest  of  the  body. 
In  some  cases,  however,  the  lips  are  paralysed  as  part  of  the  bulbar 
palsy  that  so  often  accompanies  the  spinal  disease.  A  remarkable 
case  in  which  the  face  shared  the  atrophy  of  the  limbs,  and  the  tongue 
escaped,  has  been  recorded  by  Langer  ;*  on  the  other  hand,  the  tongue 
has  been  the  first  part  to  suffer,  the  arms  being  affected  a  month 
later.f  Bulbar  paralysis,  however,  often  runs  its  course  without 
muscular  wasting  in  the  limbs,  and  when  the  latter  supervenes  it  is 
generally  quite  subsidiary.  The  like  statement  may  be  made  of  the 
converse  relation,  although  it  must  be  remembered  that  the  anterior 
nerve-cells  and  those  of  the  bulbar  nuclei  are  analogous  structures, 
and  may  be  similarly  and  even  simultaneously  affected. 

As  the  wasting  progresses,  the  appearance  of  the  parts  in  which  it 
is  most  advanced  becomes  extremely  changed.  All  trace  of  muscle 
may  disappear  from  parts  of  limbs  or  even  from  an  entire  limb 
(Fig.  144) ;  and  there  is  usually  a  wasting  of  the  adipose  tissue  as  weU 
as  of  the  muscle,  so  that  the  bone  seems  to  be  covered  only  by  fascia 
and  skin.  The  transverse  processes  of  the  vertebrae  may  be  felt  in 
the  hollow  beside  the  spine,  and  the  bony  prominences  about  the 
shoulder  may  be  almost  as  conspicuous  as  in  a  skeleton,  so  that 
beneath  the  acromion  there  may  be  a  groove,  into  which  the  finger 
can  be  placed,  in  consequence  of  the  descent  of  the  head  of  the  hujnerus 
from  the  glenoid  cavity.  The  unequal  affection  of  antagonistic 
muscles  leads  to  various  contractions  and  deformities.  In  the  hand, 
especially,  distortion  is  apt  to  occur ;  from  preponderant  paralysis  of 
the  interossei  and  contraction  of  the  long  flexor  and  extensor  muscles, 
*  Vienna  Medical  Society,  March  17th,  1882. 
t  Biidsall,  '  Journal  of  Nerv.  and  Ment.  Dis.,'  1887,  xiv,  p.  256. 


OEEONIO    MUSCULAR  ATEOPHT.  539 

the  **  claw-Hire  hand "  develops  in  extreme  degree  (Figs.  19  and  20, 
p.  43).  If  all  the  muscles  of  a  part  are  equally  and  simultaneously 
affected,  no  deformity  results  unless  an  unsupj^orted  part  yields  to 
the  influence  of  gravitation  (Fig.  144).  Lordosis  is  very  common 
in  cases  in  which  the  trunk  and  hip  muscles  are  involved.  It  is  often 
the  indirect  result  of  the  weakness  of  the  extensors  of  the  hip- joint. 
It  is  produced  in  the  manner  described  in  the  account  of  pseudo- 
hypertrophic paralysis,  in  which  it  is  almost  constant. 

The  electrical  irritability  of  the  wasted  miiscles  presents  changes 
which  vary  in  character  in  diiferent  cases.  When  the  wasting  is  slow, 
there  is  usually  a  diminution  in  both  faradic  and  voltaic  irritability, 
similar  in  character  to  each  current.  But  the  amount  of  contraction 
that  can  be  produced  is  far  less  than  normal,  and  progressively  lessens. 
The  irritability  fails  with  the  muscular  nutrition,  and  when  the 
wasting  is  great,  only  a  slight  contraction  can  be  obtained,  even  with 
a  strong  current.  When  the  wasting  is  extreme,  irritability  at  last 
becomes  extinct,  but.  the  voltaic  irritability  of  the  musciilar  fibres 
persists  long  after  the  faradic  u'ritability  has  disappeared.  It  may 
be  normal  in  degree,  or  lowered,  but  is  seldom  increased  in  such  cases. 
The  quality  of  the  voltaic  irritability  may  also  be  normal,  but  some- 
times ACC  (anodal  closure  contraction)  occurs  as  readily,  or  more 
readily,  than  KCC.  Tetanic  contraction,  during  the  passage  of  the 
current  is  produced  with  undue  readiness  compared  with  the  closure 
contractions,  and  opening  contractions  are  often  caused  by  currents 
but  little  stronger  than  those  that  cause  closure  contractions.  Thus 
there  may  be  the  qualitative  change  of  the  reaction  of  degeneration 
without  the  voltaic  increase  (see  p.  74).  When  there  is  the  rapid  and 
considerable  paralysis  described  on  p.  644,  either  at  the  onset  or 
during  the  course  of  the  disease,  the  palsy  being  followed  by  rapid 
wasting,  there  may  be  quick  extinction  of  faradic,  with  actual  exalta- 
tion of  voltaic  irritability,  and  the  reaction  of  degeneration  may  be 
present  in  all  its  characteristics.  Between  these  forms  every  inter- 
mediate condition  may  be  met  with,  including  the  mixed  reaction 
described  on  p.  32. 

The  mechanical  irritability  of  the  muscles  is  considerably  increased ; 
a  tap  causes  a  local  contraction  of  the  fibres  struck.  Moreover, 
spontaneous  flickering  contractions  of  parts  of  the  muscles  are  very 
common,  now  of  one  bundle,  now  of  another,  conspicuous  to  the  eye, 
although  scarcely  felt  by  the  patient.  This  "  fibrillation,"  as  it  is 
termed,  is  not  invai-iable,  nor  is  it  confined  to  this  disease.  It  may 
sometimes  be  observed  in  muscles  that  are  not  yet  invaded  by  the 
wasting,  but  where  it  is  observed,  in  this  disease,  atrophy  usually 
follows. 

In  the  parts  affected  by  the  characteristic  wasting,  all  reflex  action  is 
abolished,  clearly  in  consequence  of  the  damage  to  the  motor  part  of 
the  reflex  arc  ;  the  afferent  portion  of  the  arc  is  unaffected,  for,  as  we 
shall  see,  there  is  no  loss  of   sensation.      The  myotatic  irritability 


540  SPIIJAL   CORD. 

("tendon-reflex  action")  is  also  lost,  and  lost  early.  The  knee-jert, 
for  instance,  disappears  as  soon  as  there  is  even  a  slight  diminution  in 
the  bulk  of  the  thigh  muscles,  in  cases  in  which  the  legs  are  the  seat 
of  primary  wasting.  The  muscles  are  flaccid  and  toneless — a  condition 
that  may  be  termed  "  atonic  atrophy."  To  this  common  rule,  however, 
exceptions  are  sometimes  met  with  ;  there  may  be  from  the  first  rigidity 
of  the  affected  muscles.  When  this  is  the  case,  the  wasting  does  not 
go  on  to  the  entire  destruction  of  the  muscle ;  often  it  is  considerable, 
but  not  extreme,  and  it  may  be  trifling.  "With  this  rigidity  there  is  a 
preservation  or  even  increase  of  myotatic  irritability.  We  may  term 
this  "tonic  atrophy."  It  is  generally  associated  with  spastic  para- 
lysis, without  wasting,  in  some  other  part.  For  instance,  in  one  case 
the  forearms  and  hands  were  the  seat  of  this  tonic  atrophy,  and  in  the 
legs  there  was  simple  spastic  paraplegia .  We  shall  presently  consider 
the  pathology  of  this  condition.* 

Sensory  symptoms  are  always  subordinate  and  usually  very  slight. 
They  are  confined  to  pain,  usually  dull  and  rheiunatoid  in  character, 
felt  chiefly  in  the  limbs  in  which  the  malady  is  most  active.  Such 
pains  are  common  at  the  onset,  as  we  have  already  seen,  and  they  may 
recur  from  time  to  time  during  the  course  of  the  disease.  Sometimes 
vague  feelings  of  "  numbness "  or  "  deadness  "  are  complained  of, 
but  cutaneous  sensibility  is  never  impaired.  (When  anaesthesia  accom- 
panies muscular  wasting,  both  symptoms  are  due  either  to  chronic 
meningitis,  damaging  the  nerve-roots,  or  to  disseminated  focal  myelitis, 
syringomyelia,  or  multiple  neuritis.)  Nor  do  the  muscles  lose  such 
sensibility  as  they  normally  possess.  Perception  of  posture  persists, 
and  the  muscles  continue  sensitive  both  to  pressure  and  to  exten- 
sion. 

When  the  arms  are  the  seat  of  such  atrophy  as  has  been  described, 
the  legs,  if  not  also  wasted,  may  be  normal,  or  they  may  be  slightly 
affected,  the  thighs  thin,  and  the  knee-jerks  lost.  Often  they  are  the 
seat  of  gradual  loss  of  power  without  wasting,  and  with  an  increase  of 
myotatic  irritability.  The  knee-jerk  is  excessive ;  a  clonus  can  readily 
be  obtained,  and  the  reflex  over-action  of  the  muscles  may  ijicrease  to 
actual  rigidity  and  spasm,  so  that  there  is  the  condition  of  spastic 
paraplegia  described  in  a  preceding  chapter.  It  is  rare,  however,  for 
the  spasm  to  reach  the  higher  degree  of  intensity,  and  for  the  muscles 
to  present  the  massive  firmness  that  characterises  the  simple  form  of 
spastic  paraplegia.  On  the  other  hand,  there  is  often  some  diminu- 
tion in  the  size  of  the  muscles,  and  thus  we  have  a  gradation  to  the 
condition  of  tonic  atrophy  just  described.    In  this  condition,  moreover, 

*  It  should  be  noted,  however,  that,  in  the  muscles  that  are  the  seat  of  toneless 
wasting,  when  the  atrophy  has  attained  an  extreme  degree,  slight  rigidity  may 
develop,  accompanied  by  tenderness  of  the  muscle.  It  is  probable  that  this  rigidity 
is  myopathic,  and  due  to  the  structural  changes  in  the  muscles  presently  to  be 
described.  This  condition  is  rare :  the  early  flaccidity  is  usually  maintained  to  the 
last. 


CHEONIC   MUSCULAR   ATliOPHY.  541 

there  is  usually  only  a  moderate  diminution  in  electrical  excitability, 
■which  does  not  go  on  to  extinction.  The  one  condition  does  not  seem 
to  pass  into  the  other ;  the  state  of  rigidity  and  myotatic  excess  does 
not  give  place  to  muscular  relaxation ;  atonic  atrophy  does  not  succeed 
the  tonic  wasting.  In  very  rare  cases,  of  which  I  have  seen  a  few 
instances,  there  is  a  similar  condition  in  the  hands  when  the  shoulders 
are  the  seat  of  simple  atonic  atrophy.  The  forearms  are  then  rigid^ 
moderately  wasted,  with  myotatic  excess,  while  the  muscles  of  the 
shoulders  are  extremely  wasted  and  absolutely  flaccid.  In  still  more 
rare  cases  the  whole  arms  are  thus  affected,  are  thin  and  rigid,  and  in 
no  part  is  there  atonic  atrophy.  In  one  remarkable  case  of  this 
character,  the  muscles  of  the  trunk  participated  in  the  spasm ;  after 
coughing  or  yawning,  respiration  would  be  checked  for  a  few  seconds 
by  general  spasm,  and  whenever  the  patient  was  raised  from  the  bed, 
the  arms,  legs,  back  and  neck  became  stiff  in  tetanoid  rigidity,  the  head 
being  bent  backwards. 

The  combinations  of  the  ordinary  atonic  wasting,  and  loss  of  power 
without  wasting  and  with  increased  myotatic  irritabihty,  are  exceed- 
ingly numerous  and  variable.  The  most  frequent,  as  ah'eady  stated, 
is  flaccid  atrophy  in  the  arms  and  spastic  paralysis  in  the  legs ;  but  I 
have  twice  seen  atonic  atrophy  below  the  knees,  with  weak  but 
•well-nourished  thigh  muscles  and  much  increased  knee-jerk.  The 
variable  combination  of  these  conditions  gives  rise  to  differences  that 
are  more  apparent  than  real,  and  may  easily  be  allowed  undue  weight 
in  classification.     Their  explanation  will  be  considered  presently. 

The  extreme  emaciation  of  the  most  affected  -parts  shows  that  the 
adipose  tissue  wastes  as  well  as  the  muscles.  Indeed,  the  sufferers 
usually  become  generally  thin,  often  to  an  extreme  degree.  The 
atrophied  limbs  are  usually  cold,  and  may  be  livid  or  pale,  but  there  is 
no  tendency  to  acute  vaso-motor  disturbance  in  the  ordinary  form  of 
the  disease.  I  have  known  the  skin  of  the  face  to  become  thin  and 
smooth,  so  that,  in  one  instance,  the  dark  iris  could  be  seen  through 
the  closed  eyelids,  but  such  a  change  is  quite  exceptional.  In  another 
patient  there  was  a  very  remarkable  form  of  local  atrophy.  Certain 
areas  of  muscles  underwent  wasting,  the  rest  being  normal ;  the  aifec- 
tion  commenced  by  a  livid  discoloration  of  the  skin,  and  the  wasting 
seemed  to  involve  the  skin,  subcutaneous  tissue,  and  muscle,  causing 
local  depressions.  Considerable  tracts  of  the  trapezii  were  thus 
affected,  and  smaller  spots  in  the  arms  and  legs.  The  patient  was  a 
single  woman  of  thirty-five,  and  the  disease,  which  did  not  show  a 
strongly  progressive  tendency,  is  probably  a  special  affection,  perhaps 
of  the  nature  of  scleroderma. 

The  functions  of  the  sympathetic  are  not,  as  a  rule,  deranged. 
Dilatation  or  contraction  of  one  pupil  has  been  frequently  observed, 
chiefly  in  association  with  atrophy  of  the  muscles  that  are  supplied 
from  the  lower  part  of  the  cervical  region ;  and  it  no  doubt  depends 
on  the  disease  of  the  spinal  cord,  and  not  of  the  sympathetic  itself. 


542  SPINAL  COED. 

The  reflex  action  of  the  iris  is  usually  normal,  and  optic  nerve  atrophy 
never  occurs.*     Nystagmus  is  present  in  rare  cases. 

The  visceral  functions  are  usually  little  disturbed.  Sexual  power  is 
often  lost.  The  sphincters  rarely  suffer,  even  when  the  wasting  is 
general  and  extreme;  occasionally  they  are  involved,  and  they  may 
even  suffer  early.  In  other  cases,  in  which  the  legs  become  weak  after 
the  wasting  sets  in  in  the  arms,  the  affection  of  the  sphincters  may 
coincide  with  that  of  the  legs.  In  the  composition  of  the  ui'ine,  slight 
changes  have  been  found,  but  not  constantly.  Urea  has  been  increased 
in  some  cases,  lessened  in  others. f  The  quantity  of  lime  has  been 
found  to  be  increased  (Fromman),  that  of  kreatin  diminished,  even 
to  one  tenth  of  the  normal  (Rosenthal,  Langer) .  Glycosuria  has  been 
observed  in  association  with  bulbar  symptoms.  J  The  lungs  may  suffer 
from  the  impairment  of  breathing  power,  when  the  intercostals  and 
diaphragm  are  weakened. 

Varieties. — The  chief  varieties  of  the  disease  depend  on  the  relative 
distribution  of  the  three  conditions:  (1)  atonic  atrophy,  becoming 
extreme ;  (2)  muscular  weakness  with  spasm,  but  without  wasting,  or 
with  only  slight  wasting;  and  (3)  tonic  atrophy,  rarely  extreme  in 
degree,  with  myotatic  excess.  The  commonest  condition  is  to  have 
atonic  atrophy  in  the  arms  and  upper  part  of  the  trunk,  with  simple 
weakness  and  spasm  in  the  legs.  Atonic  atrophy  in  both  arms  and 
legs  is  less  common,  and  the  least  common  is  tonic  atrophy  alone,  in 
the  arms,  or  universal.  The  last  is,  indeed,  extremely  rare.  It  is 
important  to  note,  however,  that  these  conditions  may  coexist  in  every 
degree  and  combination ;  between  universal  atonic  atrophy  on  the  one 
hand,  and  universal  spastic  paralysis  without  wasting  on  the  other, 
there  is  every  gradation.  The  latter  does  not  come  into  the  category 
now  under  consideration,  but  similar  cases  are  met  with  in  which  there 
is  atrophy  of  a  few  muscles  (as,  for  instance,  of  the  hands  only),  which 
complete  the  series.  The  varieties  due  to  the  different  combinations 
of  atrophy  and  paralysis  have  been  already  alluded  to. 

A  peculiar  form  of  muscular  atrophy  has  been  mentioned,  first 

*  I  have  once  met  with  a  remarkable  reflex  fixation  of  the  eyeballs  in  a  case  of 
advanced  progressive  muscular  atrophy.  If  the  patient,  looking  to  one  side,  was 
suddenly  told  to  look  at  an  object  on  the  other  side,  his  head  was  instantly  turned 
towards  the  second  object,  while  the  eyes  remained  fixed  on  the  first,  by  a  movement 
corresponding  to  that  of  the  head  but  in  the  opposite  direction,  and  then,  after  a 
few  seconds,  they  were  slowly  moved  towards  the  second  object.  The  phenomenon 
continued  to  the  end  of  the  patient's  life.  As  I  pointed  out  in  an  account  of  this 
curious  condition  ('  Brain,*  vol.  i),  it  is  interesting  evidence  of  a  normal  reflex 
mechauiam  in  the  fixation  of  the  eyes ;  this  was,  as  it  were,  isolated  by  disease, 
which  lessened  voluntary  control  over  it. 

t  In  a  patient  who  weighed  7  st.  13  lbs.,  and  whose  urine  varied  between  760  and 
960  cubic  centimetres,  1  found  the  daily  excretion  of  urea  to  vary  between  10*7  and 
15  grammes,  the  average  being  13  grammes.  This  is  just  half  the  normal  average 
for  a  man  of  that  weight,  which  would  be  (according  to  Parkes)  24  grammes. 

X  Rovighi  and  Meiotti,  'Riv.  Sper.,'  1888,  p.  315. 


CHRONIC   MUSUULAE    ATEOPHY.  543 

conspicuous  in  the  peroneal  regions  *  and  spreading  thence  to  other 
muscles  of  the  leg  and  to  the  hands.  It  is  characterised  bj  occurrence 
in  early  life,  and  often  in  several  members  of  the  same  family,  and 
has  been  termed  the  "  peroneal  type  "  by  Dr.  H.  Tooth,  the  "  neuritic 
(neurotische)  form  "  of  Hoffmann.  It  is  probably  not  dependent  on 
the  spiaal  cord,  and  is  considered  more  fully  at  a  subsequent  page. 

Complications. — Progressive  muscular  atrophy  is  occasionally  accom- 
panied by  the  symptoms  of  some  other  degenerative  disease  of  the 
spinal  cord.  The  paralysis  with  spasm,  already  described,  can 
scarcely  be  regarded  as  a  complication :  it  is  rather  part  of  the  disease, 
and  its  relation  to  the  other  symptoms  will  be  considered  when  we 
discuss  the  pathology  of  the  affection.  In  rare  cases,  muscular  atroj^hy 
in  the  arms  is  accompanied  by  the  symptoms  of  locomotor  ataxy  in 
the  legs.  By  far  the  most  frequent  complication  is  bulbar  paralysis, 
wealniess  of  the  lips,  tongue,  pharynx,  and  often  of  the  laryngeal 
muscles.  It  is  the  expression  of  a  degenerative  process  in  the  medulla, 
similar  to  that  which,  in  the  spinal  cord,  causes  the  affection  of  the 
limbs.  It  may  come  on  at  any  stage  of  the  disease,  may  precede  the 
spinal  symptoms,  or  only  come  on  when  these  have  attained  a  con- 
siderable degree.  In  characters,  the  bulbar  palsy  may  resemble 
perfectly  that  which  occurs  in  isolated  form,  and  wiU  be  described 
among  the  diseases  of  the  brain.  Frequently,  however,  there  is  but 
little  paralysis  of  the  tongue,  even  when  swallowing  and  articulation 
are  much  impaired.  There  may  also  be  slight  interference  with  articu- 
lation when  there  is  no  marked  bulbar  palsy.  A  minor  complication 
is  the  neuralgic  pain,  which,  as  already  stated,  is  sometimes  trouble- 
some in  the  early  stage  of  cases  that  are  due  to  cold.  In  several 
patients  I  have  known  headache  to  be  frequent  and  severe  throughout 
the  course  of  the  disease.  General  paralysis  of  the  insane  has  been 
met  with  as  an  exceptional  complication. f 

Course. — The  malady,  in  most  cases,  is  steadily  progressive,  as  its 
name  implies,  but  in  the  rate  of  its  progress  it  varies  in  different  cases, 
and  iu  the  same  case  at  different  periods.  It  may,  moreover,  become 
stationary  (sometimes  as  the  result  of  treatment)  at  any  period  in  its 
course,  and  when  once  actually  arrested,  it  does  not  usually  again 
become  active.  Unfortunately,  the  tendency  to  cessation  is  greatest 
in  the  later  stages  of  the  disease,  when  there  is  little  except  life  to  be 
preserved.  Sometimes  progress  ceases  at  an  earlier  stage,  and  chiefly, 
I  think,  in  those  cases  in  which  the  atrophy  is  strictly  symmetrical, 
and  develops  simultaneously,  or  almost  simultaneously,  on  the  two 
sides.  Thus  I  have  met  with  several  cases  in.  which  there  was 
symmetrical  wasting  in  the  two  hands,  or  in  certain  muscles  of  the 
two  forearms  or  of  the  two  upper  arms,  and  in  which  the  atrophy, 

*  Cases,  however,  have  been  described  more  recently  in  which  the  upper  limbs 
suffered  first  or  simultaneously  (Hoffmann,  'Deutsch.  Zeitschr.  f.  Nervenheilk.,' 
1891,  i). 

t  Tambroni,  'Riv.  Sper.  de  Fren.,  &c.,'  1887,  xiii,  p.  184. 


644  SPINAL   COED. 

after  attaining  a  considerable  degree  in  its  limited  seat,  had  "become 
stationary  and  continued  so.  Occasionally  some  symptom  (as  weak* 
ness  of  the  sphincters)  may  pass  away,  although  the  atrophy  pro- 
gresses. 

When  the  progress  at  the  commencement  is  rapid,  it  usually  con- 
tinues rapid,  until  the  disease  has  attained  a  wide  extent,  although 
the  acute  local  onset  mentioned  below  may  be  followed  by  slow 
extension.     When  it  begins  slowly,  it  is  usually  slow  throughout. 

Although  the  disease  sometimes  commences  in  the  ?8cond  arm  very 
soon  after  its  onset,  it  more  commonly  makes  some  progress  in  its 
primary  seat  before  beginning  on  the  second  side,  and  the  interval 
that  elapses  varies  with  the  rate  of  extension.  It  often  happens  that 
a  year  intervenes  between  the  affection  of  the  two  arms,  and  I  have 
known,  in  a  very  chronic  case,  the  atrophy  to  slowly  progress  in  one 
arm  for  seven  years  before  it  showed  itself  in  the  other.  It  is  not 
common  for  the  arms  to  be  reduced  to  practical  helplessness  in  less 
than  two  or  three  years,  but  the  hands  may  become  useless  in  as  short 
a  time  as  six  months,  while  in  one  case  the  wasting,  commencing  at 
the  shoulder,  had  invaded  the  whole  arm  in  the  course  of  a  month. 
The  shortest  time  in  which  I  have  known  a  patient  to  reach  the  last 
stage  of  the  disease  is  nine  months. 

With  any  rate  of  general  progress,  the  otherwise  uniform  course  of 
tne  disease  may  be  broken  by  the  occurrence  of  almost  sudden  palsy 
in  a  certain  group  of  muscles.  Considerable  loss  of  power,  it  may  be 
absolute  paralysis,  comes  on  in  a  few  days,  or  even  in  a  few  hours. 
It  may  occur  at  the  onset  of  the  affection.  The  extensors  of  the  wrist 
and  fingers  are  the  muscles  most  commonly  thus  affected.  The  weak- 
ness is  usually  followed  by  a  well-marked  degenerative  reaction  in  the 
muscles.  I  have  seen  several  cases  which  began  by  such  subacute 
or  even  acute  paralysis  of  the  extensor  muscles,  first  in  one  arm  and 
soon  afterwards  in  the  other ;  the  initial  condition  closely  resembled 
the  paralysis  from  lead  poisoning,  even  to  the  escape  of  the  supinator 
longus  and  ext.  ossis  met.  pol.  This  cause  was,  however,  excluded,  and 
soon  the  muscles  of  the  shoulder  and  back  presented  commencing  slow 
progressive  atrophy,'  followed  by  wasting  of  the  interossei  until  a 
typical  state  was  reached.  In  another  case,  the  quick  loss  of  power 
was  confined  to  the  extensors  in  one  arm,  which  were  already  weak 
and  slightly  wasted,  most  of  the  other  muscles  of  the  upper  limbs 
having  been  long  atrophied.  When  there  is  weakness  of  the  legs 
without  wasting,  the  onset  of  this  may  coincide  with  the  atrophy  of 
the  arms,  or  raay  succeed  it  at  any  interval.  In  one  case  five  years 
elapsed,  after  the  arms  began  to  waste,  before  the  legs  became  weak. 
It  is  very  rare  for  the  paraplegic  weakness  to  occur  first. 

The  chief  danger  to  life  is  from  pulmonary  maladies,  rendered  grave 
from  the  weakness  of  the  muscles  of  respiration.  The  common  com- 
plication of  bulbar  paralysis  is  another  frequent  cause  of  death,  either 
by  the  interference  with  swallowing  and  nutrition,  or  by  the  laryngeal 


CHRONIC    MUSCULAE   ATROPHY.  545 

paralysis.    Less  commonly,  deatli  results  from  bedsores  and  septicaemia, 
or  from  intercurrent  maladies. 

Pathological  Anatomy. — The  wasting  of  the  muscles  is  as  evident 
after  death  as  during  life.  They  are  reduced  in  size  and  pale  in 
colour.  Sometimes  there  is  little  in  the  tint  of  what  remains  to 
suggest  muscular  tissue.  Parts  of  a  muscle  may  he  hardly  dis- 
ti.;<niishable  from  adjacent  fat.  On  the  other  hand,  the  bulk  of  the 
musx.^-^  may  be  dark,  and  pale  streaks  in  it  may  mark  the  position  of 
local  dbj;  ■'ueration.  Under  the  microscope  the  fibres  present  various 
changes,  aad  of  these  four  are  well  defined.  (1)  There  may  be  simple 
narrowing  of  the  fibres,  without  any  considerable  change  in  their 
striation  (Fig.  147),  although  the  striae  often  seem  to  be  further  apart 
than  normal,  and  sometimes  the  fibrillary  segmentation  is  unusually 
distinct.  (2)  Simple  fatty  degeneration,  in  which  the  transverse 
striation  gives  place  to  a  granular  appearance  (Fig.  148),  the  granules 
become  larger  and  fewer  (Fig.  147)  until  ultimately  distinct  globules 
are  scattered  through  the  sheath.  Where  the  muscle  resembles  fatty 
tissue  to  the  naked  eye,  the  microscope  may  show  only  sarcolemma 
sheaths  containing  groups  of  globides.  (3)  Muscular  fibres  are  seen 
in  which  the  sheath  contains  only  a  clear  material  enclosing  a  few 
fatty  globules,  and  a  few  transverse  striae,  faint,  as  if  fading  away.  It 
is  probable  that  this  is  not  the  result  of  fatty  degeneration,  but  of  a 
different  process,  which  has  been  termed  "  vitreous  degeneration  " — a 
sort  of  dissolution  of  the  striae,  indicated  by  the  appearance  of  such 
fibres  as  are  shown  in  Figs.  147,  148,  149.  (4)  A  longitudinal 
striation  develops  in  the  fibre,  and  at  first  co-exists  with  the  trans- 
verse striation ;  but  ultimately  the  latter  becomes  indistinct,  and  the 
fibre  looks  like  a  fasciculus  of  longitudinal  connective- tissue  fibres 
(Fig.  150).  Sometimes,  with  the  longitudinal  striation,  the  fibre 
presents  a  transverse  striation  very  much  finer  than  normal,  the  striae 
being  narrower  and  nearer  together,  as  if  from  a  division  of  the 
"  sarcous  elements."  This  change  may  sometimes  be  seen  alone,  and 
may  be  present  in  only  one  part  of  a  fasciculus.  Thus  at  one  place 
the  number  of  striae  in  ywos  i^ch  was  only  seven,  while  in  another 
region  of  the  same  fasciculus  seventeen  were  to  be  counted  in  the  same 
space,  A  tendency  to  transverse  Assuring  has  also  been  described, 
but  this  is  probably  artificial.  Fatty  globules  accumulate  between  the 
fibres  (Fig.  150),  accompanied  in  some  cases  with  granules  and  masses 
of  reddish-brown  pigment.  There  is  often  also  an  increase  of  the 
nuclei  (Fig.  148),  and  sometimes  of  the  fibres  of  the  interstitial  tissue. 
Two  or  three  rows  of  nuclei  may  lie  between  the  fibres.  The  capil- 
laries may  be  dilated  and  distended.  It  is  very  common  to  see 
muscular  fibres  that  are  much  altered  side  by  side  with  others  that 
present  a  nearly  normal  appearance.  Ultimately  the  sheaths  become 
empty  and  shrink,  and  may  be  scarcely  distinguishable  from  the 
interstitial  fibrous  tissue. 

The  peripheral  nerves  contein  many  degenerated  nerve-fibres,  and 
VOL.  I.  35 


546 


SPINAL    COED. 


the  terminal  brandies  for  tlie  muscles  a  still  larger  number.  If  the 
nerves  are  traced  up  to  tbe  cord  it  is  found  that  the  degenerated  fibres 
come  only  from  the  anterior  roots.  These  are  conspicuously  changed 
to  the  naked  eye,  small  and  grey.  They  may  resemble  fine  threads  of 
connective  tissue,  and  under  the  microscope  no  nerve-fibres  may  be 
found  in  them,  or  only  a  few  may  remain  of  normal  aspect,  the  rest 
being  in  various  stages  of  degeneration,  or  represented  only  by  their 
emx^ty  sheaths.     The  degree  of  affection  of  the  anterior  roots  corre- 


FiG.  147. 


Fm.  148. 


Fii>.  U9, 


Pig.  150. 


Fibres  of  Wasted  Muscles  ik  Pbogbessivb  Mfsculab  Ateopht. 

Fig.  147. — Narrowed  fibres  with  nearly  normal  striation;  others  clear,  containing 
a  few  fat  globules,  and  a  few  faint  transverse  strisB  and  some  longitudinal  striation; 
lar^e  globules  of  fat  lie  in  the  interstitial  tissue  in  front  of  one  narrowed  fibre. 

Fig.  148. — Gr.inular  degeneration :  afibre  with  normal  transverse  striation  presents 
also  indications  of  longitudinal  striation.     Increase  of  nuclei  of  interstitial  tissue. 

Fig.  149. — Two  normal  fibres  and  one  presenting  the  clear  homogeneous  aspect, 
with  a  few  fat  globules  and  some  faint  striation. 

Fig.  150. — Several  fibres  similar  to  that  of  the  last  figure,  with  globules  of  fat 
between  them.  On  the  rigrht  is  a  fibre  which  has  undergone  complete  longitudinal 
stiiation,  the  normal  strisB  having  disappeared,  so  that  it  resembles  the  adjacent 
interstitial  fibrous  tissue.  Other  fibres  in  the  same  muscle  were  in  intermediate  stages 
of  degeneration,  some  transverse  striation  co-existing  with  the  longitudinal  striation. 

sponds  to  the  wasting  in  the  parts  supplied  by  them.    The  posterioT 
roots  are  normal. 

The  spinal  cord  is  often  softer  than  natural  at  the  affected  part, 
and  the  white  substance  of  the  lateral  columns  may  be  grey  and 
translucent  in  aspect,  especially  in  the  cervical  enlargement.  Under 
the  microscope  morbid  changes  are  seen  in   the   anterior  comua. 


CHRONIC    MUSCULAE    ATKOPHT.  547 

and  also  in  most  cases,  in  tlie  antero-lateral  white  columns.  The 
change  in  the  anterior  comua  corresponds  in  its  intensity  to  the 
origin  of  the  nerves  to  the  most  affected  parts,  and  since  the  latter 
are  usuaUj  the  arms,  the  disease  is  generally  most   intense  in  the 

Fig.  151. 


^\ 


1^    iWloI^ 


h      \ 


1  ' 


'^ 


Fig.  152. 


Pig.  151. — A  normal  anterior  cornu  for  comparison  with  the  next  flgnre. 
Fig.  152. — Progressive  muscular  atrophy;  anterior  cornn,  cervical  region, 
rom  drawinsrs  bv  Dr.  H.  R.  Spencer.) 


ma.  152. — Jrrogressive  muscular  atrc 
(From  drawings  by  Dr.  H.  R.  Spencer.) 


cervical  enlargement.  In  stained  sections  the  grey  matter  of  the  horn 
is  less  deeply  tinted  than  normal,  but  in  some  parts  it  may  stain  more 
deeply,  especially  in  the  circumference  of  the  cornu  and  the  processes 
of  grey  matter  which  project  into  the  white  column.     Occasionally, 


548 


SPINAL   CORD. 


larger  areas  hsuve  a  dense  aspect 
and  stain  deeply,  but  the  central 
part  of  the  cornua  is  generally  pale 
and  wasted  in  aspect.  Most  of  the 
large  nerve-cells  have  disappeared ; 
many  entirely,  while  others  are  re- 
presented only  by  small  angular 
bodies.  Not  a  single  large  cell  may 
be  seen  in  a  section  which,  in  a 
normal  cord,  would  contain  a  consi- 
derable number.  Frequently,  how- 
ever, a  few  large  cells  can  still  be 
seen,  but  most  of  these  have  lost 
their  processes  and  are  more  glo- 
bular than  normal.  The  inter- 
stitial tissue  is  also  changed.  The 
nerve  fibrillse  waste  with  the  cells, 

Fio.  153. — Progressive  mnscular  atrophy; 
degeneration  of  the  anterior  coniua  and 
pyramidal  tracts.  A,  medulla  oblongatn, 
complete  degeneration  of  the  anterior 
pyramids,  A  P;  B,  at  the  upper  part  of 
the  decussation  of  the  pyramids ;  D,  the 
decussation  of  the  degenerated  fibres ; 
A  P,  the  pyramids,  still  incomplete.  C, 
cervical,  JD,  dorsal,  E,  lumbar  sections. 
The  degeneration  of  the  anterior  cornua 
18  complete  in  C,  but  in  E  a  few  cells 
remain,  for  tlie  most  part  without  pro- 
cesses, in  C  and  D  tlie  degeneration 
of  both  anterior  and  lateral  pyramidal 
tracts  is  conspicuous;  in  E  the  anterior 
tract  has  ceased,  and  the  lateral  tract 
extends  up  to  the  surface  of  the  cord, 
from  which  it  is  separated  in  C  and  D 
by  the  undegenerated  direct  cerebellar 
tract.  In  C  and  D  the  degeneration 
extends  forwards  in  front  of  the  lateral 
pyramidal  tract,  but  gradually  ceases. 
The  fibres  of  the  anterior  commissure 
are  also  degenerated.* 

•  In  this  case  the  arms  were  greatly 
wasted  and  fluccid;  the  legs  were  para- 
lysed, rigid,  with  moderate  wasting.  The 
muscles  of  the  back  were  also  atrophied. 
In  the  arms  the  weakness  and  wasting 
came  on  at  the  same  time,  in  the  right 
arm  some  time  before  the  left.  The 
shoulder  muscles  suffered  first,  but  the 
atrophy  quickly  spread,  and  was  ulti- 
mately extreme  in  the  nmscles  of  the 
shoulders,  forearms,  and  hands.  The  dis- 
ease ran  a  rapid  course j  death  occurred 
two  years  after  the  gradual  onset. 


CHRONIC    MUSCULAR   ATEOPHF. 


549 


and  there  is  an  increase  of  the 
small,  angular,  and  stellate  cells 
and  other  connective-tissue  ele- 
ments. The  larger  vessels  are 
dilated  and  surrounded  by  un- 
duly wide  spaces,  but  there  is 
no  considerable  distension  of  the 
capillaries.  The  comu  as  a 
whole  is  not  usually  changed 
either  in  size  or  shape.  Similar 
alterations  may  be  traced  through 
the  dorsal  region.  In  the  lumbar 
■enlargement  the  grey  matter  may 
be  normal,  even  when  it  is  much 
altered  in  the  cervical  region ; 
but  if  the  legs  are  wasted  there 
are  changes  similar  to  those  al- 
ready described.  When  the 
atrophy  begins  in  the  legs,  the 
disease  may  be  more  intense  in 
the  lumbar  than  in  the  cervical 
enlargement ;  but  more  often 
any  degeneration  that  exists  is 
slighter,  and  a  larger  number  of 
normal  or  slightly  changed  cells 

Fig.  154. — Progressive  muscular  atro- 
phy. A,  medulla  oblongata;  degene- 
ration of  the  anterior  pyramids,  A  p. 
B,  cervical,  C,  first  dorsal,  D,  first 
lumbar,  E,  mid-lumbar  sections.  The 
nerve-cells  have  disappeared  from 
the  anterior  cornua,  except  in  E,  in 
which  a  few  remain.  There  is  com- 
plete degeneration  of  the  pyramidal 
tracts,  anterior  and  lateral.  The  de- 
•cussation  at  the  medulla  was  unequal 
in  this  case;  of  the  left  pyramid 
fewerfibres  crossed  tlian  of  the  riglit, 
and  hence  the  left  anterior  pyramidal 
tract  is  larger,  while  the  right  lateral 
tract  is  smaller  than  normal,  and, 
moreover,  has  ceased  at  tlie  middle  of 
the  lumbar  enlargement,  to  which 
the  left  anterior  tract  extends.* 


*  The  patient  was  a  man  forty-eight 
years  of  age.     The  disease  followed  a 

severe  exposure  to  cold.     The  wasting  "^^^i     J      I  / 

of  the  arms  was  extreme,  and  they  were  ^^'V   1 

absolutely  powerless.     The  legs  were  ^^^■^^>^»— -JsJl—— "''^^ 

much  less  wasted  than  the  arms,  but  very  weals,  the  loss  of  power  being  out  of 
proportion  to  the  wasting. 


550  SPINAL   COKU. 

can  be  seen.  Sometimes  certain  groups  of  cells  are  little  affected 
while  others  are  mucli  atrophied.  The  degeneration  may  involve  to 
some  extent  the  intermediate  grey  matter  between  the  cornua,  but 
here  it  ceases ;  the  posterior  horn  is  always  normal. 

There  is  distinct  degeneration  of  the  anterior  root-fibres  passing 
from  the  cornu  through  the  anterior  column,  A  few  fibres  may 
remain,  but  whole  fasciculi  appear  to  be  replaced  by  fibrous  tissue. 
There  is  also  degeneration  of  the  fibres  of  the  anterior  commissure,  in 
consequence  of  which  it  stains  much  more  deeply  than  normal,  in  the 
regions  in  which  the  grey  matter  is  considerably  diseased. 

In  the  white  columns  there  is  usually  considerable,  and  often  almost 
complete  degeneration  of  the  pyramidal  tracts,  anterior  and  lateral,* 
The  area  of  sclerosis,  resulting  from  the  degeneration  of  the  two 
tracts,  varies  in  extent  according  to  the  size  of  the  anterior  tract,  and 
to  the  distance  which  it  extends  down  the  cord.  In  Fig,  154  this  is 
illustrated  in  an  unusual  manner,  in  consequence  of  an  inequality  in 
the  decussation  of  the  pyramids  at  the  medulla,  fewer  fibres  of 
the  left  pyramid  having  crossed  than  of  the  right,  so  that  the  left  half 
of  the  cord  contains  more  than  its  proper  share  of  the  fibres,  and  the 
anterior  tract  extends  into  the  lumbar  region  even  further  than  does 
the  lateral  tract.  Where  the  direct  cerebellar  tract  exists,  this,  always 
unaffected,  limits  the  sclerosis  of  the  lateral  tract  on  the  outer  side. 
On  the  inner  side,  the  "  lateral  limiting  layer  "  (see  p.  221)  is  usually 
much  less  degenerated  than  the  pyramidal  tract,  and  intervenes  be- 
tween the  latter  and  the  grey  matter  ;  but  this  layer  suffers  in  some 
degree,  perhaps  by  the  affection  of  outlying  pyramidal  fibres  con- 
tained in  it.  The  sclerosis  does  not  usually  cease  at  the  anterior 
extremity  of  the  pyramidal  tract,  but  extends  forward  in  the  "  mixed 
zone"  of  the  lateral  column,  lessening  in  intensity,  and  ceasing 
usually  opposite  the  outer  part  of  the  anterior  cornu.  It  is  here 
most  intense  close  to  the  grey  substance,  and  ceases  before  it  reaches 
the  surfare  of  the  cord,  the  antero-lateral  ascending  tract  being 
unaffected.  This  sclerosis  may  be  due  to  the  degeneration  of 
the  short  vertical  fibres  that  pass  between  adjacent  regions  of  the 
anterior  cornu,  and  which  share  the  degeneration  of  the  nerve-cells. 
Occasionally  it  extends,  in  slighter  degree,  into  the  anterior  columns. 
The  posterior  columns  are  always  free  from  definite  degeneration,  but 
there  may  be  some  general  increase  of  connective  tissue  throughout 
the  whole  cord.     The  degeneration  of  the  pyramidal  tracts  is  usually 

*  I  have  not  yet  met  with  a  single  ease  of  progressive  muscular  atrophy  in  which 
the  pyramidal  tracts  were  unaffected.  Such  cases  are  extremely  rare.  One  has  been 
published  by  Stiiimpell  ('Zenker's  Zeitsch.,'  1887,  and  'Neur.  Centralbl.,'  1888, 
p.  139)  which  was  ciiaracterised  by  the  general  wasting  of  the  arm,  a  very  slow 
course,  and  the  absence  of  leg  symptoms.  The  spinal  nerve-cells  and  motor  nerves 
were  degenerated.  Other  cases  have  been  described  by  Villiers  ('Journ.med.de 
Bruxelles,'  1897)  and  by  Beevor  in  a  communication  to  the  Neurological  Society  of 
London  in  1896. 


CHRONIO    MUSCULAR  ATROPHY.  551 

considerable  in  degree ;  often  most  of  their  fibres  seem  to  have  perished. 
If  it  is  incomplete  in  the  cervical  region,  it  may  be  slight  in  the  lower 
part  of  the  cord,  the  degenerated  fibres  being  chiefly  those  that  end 
in  the  cervical  enlargement  and  dorsal  region.  "When  the  sclerosis  is 
intense  it  is  not  confined  to  the  cord ;  it  can  be  traced  up  through  the 
decussation  (Fig.  153,  b)  and  through  the  medulla  oblongata,  in  which, 
as  Figs.  153  and  154  show,  the  whole  of  both  pyramids  may  be  degene- 
rated, so  that  they  stain  deeply,  and  no  nerve-fibres  can  be  dis- 
tinguished in  them.  The  degeneration  of  the  pyramids  has  been 
found  in  several  cases  to  extend  through  the  pons  and  crus  to  the 
internal  capsule,  and  even  through  the  white  substance  to  the  cortes. 
Above  the  capsule,  however,  the  pyramidal  fibres  are  so  mingled  with 
others  that  their  degeneration  is  no  longer  recognisable  by  the  deeper 
staining  of  a  definite  tract ;  but  it  has  been  detected  by  the  presence 
of  abundant  products  of  degeneration  in  the  path  of  the  fibres.  In 
the  motor  cortex  the  large  ganglion-cells  have  been  found  fewer  than 
normal  and  distinctly  degenerated,  many  having  lost  their  processes ; 
and,  in  the  interstitial  tissue,  the  spider-cells  and  other  connective- 
tissue  elements  are  increased  in  number.*  But  the  degeneration  of 
the  pyramidal  tracts  has  also  been  found  to  cease  at  the  crus  f  and  at 
the  decussation.  J 

In  cases  in  which  the  symptoms  of  bulbar  paralysis  were  present 
during  life,  the  motor  nuclei  of  the  medulla  have  presented  changes 
corresponding  to  those  of  the  grey  matter  of  the  spinal  cord,  with  a 
similar  degeneration  of  the  nerve-cells.  In  other  instances  the 
degeneration  of  these  nuclei  has  been  slight,  but  in  such  cases  the 
degeneration  of  the  pyramids  has  been  intense,  and  doubtless  involved 
the  fibres  connecting  these  nuclei  with  the  cortex  of  the  brain. 

The  sympathetic  nerves  and  ganglia,  when  examined,  have  been 
found  normal,  or  have  only  presented  such  changes  as  are  common 
apart  from  symptoms  of  disease  of  the  nervous  system. 

Pathology. — The  theories,  according  to  which  the  disease  was 
regarded  as  primarily  one  of  the  muscles  or  of  the  sympathetic  nerves, 
have  now  only  an  historical  interest.  The  constancy  of  the  changes 
in  the  ganglion-cells  of  the  spinal  cord,  the  degeneration  of  the  motor 
root-fibres,  and  the  analogous  effects  of  acute  lesions  of  the  anterior 
cornua,  leave  no  doubt  of  the  relation  of  the  muscular  wasting  to  the 
disease  of  the  grey  matter,  and  essentially  to  that  of  the  ganglion-cells, 
and  the  fibres  proceeding  from  them.  The  slowness  of  the  change 
causes  the  impairment  of  the  nutrition  of  the  muscular  fibres  to  pro- 
ceed pari  passu  with  that  of  the  nerve-elements,  and  the  interference 
with  motor  conduction  to  be  proportioned  to  both.  We  are  thus  able 
to  understand  the  condition  of   electrical  excitability,  and  its   slow 

*  Koschewnikoff,  'Arcliives  de  Neurologie,'  1883,  No.  18;  aud  '  Ceiitralbl.  f. 
Nerveukr.,'  1885,  p.  409 ;  Marie,  '  Neiir.  Ceutialbl.,*  1884,  p.  61. 

t  Rovighi  and  Melotti,  loc.  cit.;  Dornbluth,  'Neur.  Centialbl.,'  1889,  p.  377. 
j  Moeli,  'Arch.  f.  Psych.,*  x,  718. 


552  SPINAL   COED, 

failure,  as  nerve  and  muscle  degenerate  together.  It  is  only  wlien  tlie 
slow  degeneration  is  varied  by  a  more  acute  process  of  destruction  of 
cells  and  more  rapid  degeneration  of  fibres  that  the  muscular  tissue  ia 
for  a  long  time  less  damaged  tban  the  nerve-fibres,  and  presents 
paralysis  in  excess  of  the  wasting,  with  a  voltaic  irritability  in  excess 
of  the  faradic  irritability  of  the  nerve-endings  (p.  30).  Thus  the 
essential  lesion  of  the  disease  is  a  slow  decay  of  the  lower  segment  of 
the  motor  path,  the  segment  which  consists  of  the  ganglion- cells  and 
their  prolongations  in  the  axis-cylinders  of  the  nerve-fibres  (seep.  213). 
To  this  the  conspicuous  symptom,  the  muscular  wasting,  is  secondary. 
It  is  perhaps  better  thought  of  as  a  degeneration  of  the  whole  segment 
than  as  simply  a  lesion  of  the  ganglion-cells,  although  the  latter 
being  the  element  on  which  the  nutrition  of  the  segment  depends,  the 
two  views  are  merely  different  modes  of  stating  the  same  fact.* 

But  the  disease  is  rarely  limited  to  the  lower  segment  of  the 
motor  path.  The  cases  mentioned  in  the  note  on  p.  550  are  an 
example  of  such  rare  limitation — an  "  exception  that  proves  the  rule."t 
We  have  seen  that  the  pyramidal  tracts  are  commonly  degenerated, 
and  it  is  probable  that  the  degeneration  often  extends  throughout  their 
entire  extent,  and  involves  the  motor  cells  of  the  cortex.J  Thus  the 
upper  segment  is  often  degenerated  as  well  as  the  lower  segment.  In 
the  presence  of  that  complete  degeneration  of  the  lower  segment 
which  causes  the  atonic  atrophy  of  the  muscles,  the  degeneration  of 
the  upper  segment  can  cause  no  symptoms.  The  loss  of  power 
that  it  would  produce  is  also  caused  by  the  degeneration  of  the  lower 
segment,  and  the  latter  abolishes  the  myotatic  irritability,  excess  of 
which  is  the  characteristic  indication  of  disease  of  the  upper  segment. 
Hence  the  degeneration  of  the  upper  segment  of  the  motor  path  for 
the  muscles  that  present  the  atonic  atrophy  cannot  as  a  rule  be  recog- 
nised during  life,  for  it  cannot  produce  its  characteristic  symptoms. 

What  is  the  relation  between  the  degeneration  of  the  two  segments, 
between  the  lateral  sclerosis  and  the  affection  of  the  ganglion-cells  ? 
We  cannot  assume  (as  some  have  been  inclined  to  do)  that  the  affection 
of  the  upper  segment  is  secondary  to  that  of  the  lower,  because  a 

*  In  a  few  anomalous  cases,  degeneration  of  the  motor  cells  of  the  cord  has  been 
met  with  when  no  degeneration  could  be  detected  in  the  peripheral  mixed  nerves 
(see  Kronthal,  'Neur.  Centralbl.,'  1891,  p.  133).  The  interpretation  of  these  cases 
is  at  present  uncertain.  Possibly  complete  disappearance  of  some  fibres  left  the 
rest  apparently  normal,  although  the  skill  of  the  investigators  renders  this  explana- 
tion difficult  to  accept.  Many  more  observations,  however,  are  needed  to  justify  a 
modification  of  current  views. 

t  Moreover  the  fact  that  these  cases  may  not  be  primarily  spinal  must  be  borne 
in  mind. 

X  At  the  same  time  we  have  seen  (p.  255)  that  the  nutritional  stability  of  nerve- 
fibres  is  less  in  their  lower  than  in  their  upper  parts,  and  that  their  isolated  degene- 
ration may  be  greatest  in  the  lower  parts.  That  tliis  may  sometimes  also  be  the 
case  in  these  pyramidal  fibres  is  suggested  by  the  fact  already  stated  that  the 
degeneration  may  extend  no  higher  than  the  decussation  of  the  crura  cerebri. 


CHRONIC   MUSCULAR   ATROPHY.  553 

primary  lesion  of  the  grey  matter,  sucli  as  occurs  in  polio-myelitis, 
does  not  cause  ascending  degeneration  of  the  related  pyramidal  fibres. 
Moreover,  even  a  complete  interruption  of  the  pyramidal  tracts  is 
followed  by  no  degeneration  of  their  upper  parts.  Hence  the  iatense 
degeneration  met  with  in  progressive  muscular  atrophy  cannot  be 
regarded  as  secondary.  Neither,  when  there  is  atonic  atrophy,  can 
we  consider  the  affection  of  the  upper  segment  to  be  the  primary  lesion, 
and  to  be  the  cause  of  that  in  the  lower.  In  the  seat  of  slight  atrophy 
there  are,  as  a  rtde,  no  indications  of  the  preceding  degeneration  of 
the  upper  segment,  which  would  certainly  exist  if  this  lesion  were  1  he 
primary  change.  Moreover  degeneration  of  the  upper  segment  d()es 
not  necessarily  cause  any  degeneration  of  the  lower.  It  is  a  matter  of 
€very-day  observation  that  intense  degeneration  of  the  termination  of 
the  upper  segment  may  occur,  from  dorsal  myelitis,  for  instance,  with- 
out any  considerable  wasting  of  the  legs,  and  such  degeneration  has 
never  been  known  to  excite  the  complete  degeneration  of  the  lower 
segment  which  causes  atonic  atrophy.  Hence  the  only  adequate 
explanation  of  the  facts  is  that  the  degeneration  of  the  upper  and 
lower  segments  is  simultaneous,  or  if  not  simultaneous,  at  least  so  far 
independent  that  neither  is  the  cause  or  consequence  of  the  other ; 
both  are  the  results  of  the  same  tendency  to  degeneration  of  the 
motor  path.*  Atonic  muscular  atrophy  is  thus,  at  least  in  many 
■cases,  the  visible  expression  of  a  tendency  to  decay  of  the  whole  motor 
path  from  the  cortex  of  the  brain  to  the  muscles. 

The  weakness  of  some  parts,  as  the  legs,  with  excessive  myotatic 
irritabihty,  often  going  on  to  spasm,  is  explained  by  the  degeneration 
of  the  pyramidal  fibres  for  the  legs,  the  lower  segment  being  unaffected. 
It  is  easy  to  understand  that  the  affection  of  the  upper  segment,  and 
escape  of  the  lower,  or  the  affection  of  both,  may  vary  much  in  relative 
extent,  and  give  rise  to  ^he  multiform  varieties  already  mentioned.  In 
such  a  case  the  nerve-cells  for  the  part  thus  paralysed  are  normal. 
This  weakness  of  the  legs  usually  succeeds  the  wasting  in  the  arms, 
and  in  most  cases  in  which  spastic  paraplegia  is  followed  by  atrophy 
in  the  arms,  the  latter  have  not  shared  the  spastic  palsy.  When  this 
condition  is  associated  with  sUght  wasting  of  the  legs,  without  con- 
siderable change  in  electrical  irritability,  the  condition  exists  that  we 
have  more  than  once  considered,  in  which  we  must  assume  that  the 
motor  nerve-cells  of  the  cord,  while  structurally  intact,  undergo  sKght 
•changes  in  nutrition.  In  this  condition  there  are  many  nerve-cells  of 
nonnal  appearance  in  the  grey  matter ;  the  alterations  in  nutrition  are 
too  slight  to  cause  changes  in  aspect,  or  if  there  are  such  changes  we 
have  not  yet  learned  to  detect  them.  They  are  perhaps  results  of  the 
degenerative  changes  in  the  termination  of  the  upper  segment ;  when 
such  degeneration  is  secondary  to  a  focal  lesion  of  the  cord  or  brain, 
the  changes  in  nutrition  of  the  cells  seldom  attain  such  a  degree  as  to 

*  See  case  published  by  Mott     Brain,'  1895. 


554  SPINAL    COliD. 

arrest  tlie  myotatic  irritability,  or  to  cause  wasting  such  as  attends  the 
destruction  of  these  cells. 

In  some  cases,  however,  the  muscular  wasting  may  be  great,  although 
the  increase  of  myotatic  irritability  persists.  In  these  cases,  as  w© 
have  seen,  there  is  considerable  rigidity  of  the  muscles  throughout  the 
whole  course  of  their  wasting,  the  condition  that  we  have  termed 
"tonic  atrophy,"  In  such  a  condition  it  is  common  to  find  that  many 
nerve-cells  have  disappeared  or  are  very  small,  but  others  remain 
normal  or  slightly  changed  in  aspect.  Apparently,  in  addition  to 
the  degeneration  of  the  upper  segment  and  to  the  nutritional  changes 
just  mentioned,  we  have  then  a  considerable  degeneration  of  many, 
but  not  destruction  of  all,  the  elements  of  the  lower  segment.  We 
cannot  regard  this  as  simply  secondary  to  the  degeneration  of  the 
upper  segment,  for  the  reasons  already  given.  It  must  be  the 
expression  of  a  distinct  pathological  tendency  similar  to  that  which 
elsewhere  causes  the  atonic  atrophy  and  total  wasting,  but  slighter 
in  extent  and  later  in  time — insufficient  to  prevent  the  less  affected 
cells  from  causing  rigidity  under  the  influence  of  the  degeneratioa 
of  the  upper  segment.  It  is  doubtful  whether  the  tonic  atrophy  ever 
goes  on  to  atonic  atrophy.  Theoretically  conceivable,  it  is  certain 
that  if  it  ever  occurs  it  is  extremely  rare.  Nor  does  it  seem  that  atonic 
atrophy  ever  gives  place  to  tonic  atrophy  with  excessive  myotatic 
irritability.  The  rigidity  of  tonic  atrophy  is  due  to  the  degeneration 
of  the  upper  segment,  but  the  effect  is  not  produced  if  the  lower 
segment  is  already  the  seat  of  such  extensive  degenerative  changes  as 
abolish  myotatic  irritability.  The  pyramidal  fibres  for  the  parts  that 
are  the  seat  of  atonic  atrophy  are  constantly  found  degenerated, 
although  the  muscles  have  been  flaccid  to  the  last.  In  the  rare  cases 
(mentioned  on  p.  540)  in  which  muscles  with  atonic  atrophy  become 
rigid  towards  the  end  of  the  process,  it  is  probable,  as  the  tenderness 
suggests,  that  the  rigidity  is  idiopathic,  due  to  the  changes  in  the 
muscles,  and  is  not  dependent  on  the  ceotral  nervous  system.  It  may 
be  the  result  of  the  increase  in  the  interstitial  connective  tissue,  and 
the  longitudinal  division  and  fibrillation  by  which  the  muscular 
fasciculi  come  to  resemble  bundles  of  connective-tissue  fibres.  It  is 
also  possible  that  similar  idiopathic  muscular  changes  may  ultimately, 
in  tonic  atrophy,  maintain  and  increase  the  rigidity  that  is  primarily 
dependent  upon  the  spinal  cord. 

It  has  been  mentioned  that  the  cases  in  which  the  legs  present 
the  simple  palsy  and  spasm,  or  the  tonic  atrophy,  which  indicate 
degeneration  of  the  pyramida.1  tracts,  have  been  separated  by  Charcot, 
and  termed  "  amyotrophic  lateral  sclerosis,"  the  separation  being  based 
on  the  assumption  that  in  such  cases  the  primary  lesion  is  the  degene- 
ration of  the  pyramidal  tracts,  and  that  the  affection  of  the  grey 
matter  is  secondary  or  "  deuteropathic,"  even  where  the  atrophy  is 
atonic.  We  have  seen  also  that  this  assumption  is  unwarranted  so  far 
as  the  atonic  atrophy  is  concerned.     It  is  probable  that  the  pyramidal 


CHRONIC   MUSCULAR   ATROPHY.  556 

tracts  are  degenerated,  if  not  constantly,  at  any  rate  in  such  a  very 
large  propoi-tion  of  the  cases  of  progressive  muscular  atrophy,  that 
Charcot's  distinction  is,  in  effect,  giving  a  new  name  to  an  old  disease, 
and  that  the  sequence  is  not  that  indicated  by  the  name.  "Whether 
there  are  indications  of  lateral  sclerosis  or  not  depends  on  the 
circumstance  whether  the  degeneration  of  the  pyramidal  fibres  is  or  is 
not  more  extensive  than  the  complete  degeneration  of  the  nerve-cells 
that  causes  atonic  atrophy.  If  the  latter  is  universal  the  pyramidal 
tracts  may  be  totally  degenerated,  and  yet  there  may  be  none  of  the 
characteristic  indications  of  such  degeneration.  On  the  other  hand, 
both  arms  and  legs  may  be  the  seat  of  the  spastic  paralysis  that 
indicates  pyramidal  degeneration,  and  atonic  atrophy  may  be  limited 
to  a  few  muscles  of  the  hands.  Between  these  we  have  every  grada- 
tion, in  degree  and  distribution,  of  atonic  atrophy,  spastic  paralysis, 
and  tonic  wasting. 

The  process  in  the  grey  matter  has  been  regarded  by  some  as  a 
chronic  inflammation.  The  occasional  rapid  increase  in  the  symptoms 
may  be  thought  to  be  justification  for  this  view ;  but  the  process  in 
genera]  is  at  the  degenerative  extremity  of  the  series  of  nerve  lesions. 
The  principle  involved  in  this  question  has  been  already  discussed, 
and  we  have  seen  that,  whatever  be  the  nature  of  the  primary  process, 
we  must  recognise  secondary  tissue  changes  of  independent  energy, 
and  that  a  distinct  process  of  inflammation  may  occasionally  form  part 
of  these  (see  p.  443).  The  significance  of  the  occurrence  of  inflamma- 
tion may  therefore  easily  be  overrated,  so  far  as  concerns  the  question 
of  the  process,  and  the  tissue-elements  in  which  the  disease  begins. 

Diagnosis. — The  simultaneous  and  gradual  onset  of  weakaess  and 
wasting,  the  slow  but  progressive  increase  and  extension  of  the 
symptoms,  render  the  diagnosis  of  the  developed  malady  simple  and 
easy.  At  the  onset,  when  only  a  single  muscle  or  group  of  muscles  is 
affected,  the  question  arises  whether  the  atrophy  is  local  or  is  the 
commencement  of  a  wider  affection.  Local  atrophy  is  said  sometimes 
to  occur  from  great  over-use  of  a  muscle,  especially  one  of  the  small 
muscles  of  the  hand ;  but  such  a  cause  is  extremely  rare,  and  only  to 
be  suspected  on  the  clearest  indications. 

As  a  rule,  the  local  atrophy  from  which  the  affection  has  to  be  dis- 
tinguished is  that  due  to  disease  of  the  nerves.  The  wasting  from 
disease  of  single  nerves  or  at  a  plexus  (as  the  brachial)  is  sufficiently 
distinguished  by  its  limitation,  coupled  with  its  rapid  onset  and 
associated  sensory  symptoms.  Much  more  difficult  is  the  distinction 
of  some  forms  of  multiple  neuritis.  The  difficulty  presents  itself 
chiefly  in  two  forms :  t^l)  when  the  spinal  affection  begins  as  sub- 
acute atrophic  palsy  (see  p.  544);  (2)  when  neuritis  affects  chiefly 
motor  branches,  as  in  the  arms  in  lead  poisoning,  or  in  the  legs  in 
alcoholism,  &c.  In  the  first  case  it  is  necessary  to  wait  for  signs  of 
slower  wasting  in  other  parts  than  those  first  affected  before  a 
diagnosis  can  be  made.     In  the  second,  a  careful  search  will  generaUj 


556  SriNAL   CORD. 

reveal  other   symptoms  of  neuritis,  and  a  known  canse  is  usually 
obtrusive. 

In  pachymeningitis  of  the  cervical  region,  with  considerable  damage 
to  the  nerve-roots,  the  wasting  in  the  arms  may  resemble  that  of 
progressive  muscular  atrophy,  and  there  is  often  weakness  with 
rigidity  in  the  legs ;  but  the  wasting  is  less  chronic  in  onset,  and  is 
always  accompanied  by  distinctive  sensory  symptoms, — by  acute  pains, 
and  usually  by  anaesthesia,  irregular  in  distribution.  The  same  dis- 
tinctions suffice  for  the  diagnosis  in  diseases  of  the  ne;  ve-roots  of  the 
Cauda  equina,  as  by  a  tumour.  This  may  cause  slow  wasting  in  the 
legs,  but  there  is  always  severe  pain  and  loss  of  sensibility.  Chronic 
disseminated  myelitis  may  cause  wide-spread  muscular  atrophy,  but  is 
distinguished  by  the  presence  of  symptoms  of  irregular  damage  to 
other  structures  in  the  cord.  The  diagnosis  from  syringomyelia  will 
be  considered  in  the  account  of  that  disease. 

Erom  primary  muscular  atrophy,  "  idiopathic  atrophy,"  a  variety  of 
"  muscular  dystrophy,"  as  it  has  been  termed,  the  diagnosis  is  some- 
times easy,  sometimes  very  difficult.  It  is  easy  in  the  pseudo-hyper- 
trophic  form,  and  often  also  in  the  atrophic  variety  (the  cases  in  whieh 
n.0  muscles  are  large),  on  account  of  its  characteristic  distribution,  its 
course,  the  age  at  which  it  begins,  and  the  tendency  to  affect  many 
members  of  a  family,  and  males  more  than  females — features  that  will 
be  presently  described.  Whenever  several  cases  of  muscular  atrophy 
occur  in  a  family,  or  during  childhood  or  youth,  the  probability  is 
great  that  they  are  idiopathic  and  not  spinal.  Indeed,  the  idiopathic 
form  is  to  be  suspected  whenever  muscular  atrophy  begins  under 
twenty,  unless  there  are  distinctive  spinal  symptoms.  But  cases  are 
sometimes  met  with  in  which  idiopathic  atrophy  begins  in  adult  life, 
and  the  distinction  of  such  cases  may  be  very  difficult,  and  will  be 
better  understood  after  a  perusal  of  the  account  of  that  disease. 

Peognosis. — The  progressive  character  of  the  malady  renders  the 
prognosis,  in  every  case,  grave  and  uncertain.  The  chief  guide  is  the 
observed  tendency  of  the  morbid  process,  both  the  fact  of  its  actual 
advance  and  the  energy  it  manifests.  At  the  same  time  there  is  a 
possibility  of  arrest,  greater  in  middle  life  than  in  old  age.  Some  in- 
crease of  atrophy  in  the  parts  already  affected  may  occur  for  a  short 
time  after  the  process  in  the  cord  has  ceased  to  spread — the  muscular 
wasting  going  on  until  it  corresponds  to  the  changes  in  the  nerves  that 
have  already  taken  place.  The  prospect  of  arrest  seems  to  be  greater 
in  the  cases  in  which  the  wasting  is  strictly  symmetrical  and  nearly 
simultaneous  on  the  two  sides  than  in  those  in  which  it  is  irregular, 
and  attacks  the  second  side  when  it  has  attained  a  marked  degree  in 
the  first.  Spontaneous  cessation  unfortunately  seldom  tends  to  occur 
imtil  an  advanced  stage  is  reached ;  but,  as  the  result  of  treatment, 
arrest  may  take  place  at  any  stage.  The  danger  to  life  is  chiefly  pi'o- 
portioned  to  the  interference  with  the  muscles  of  respiration,  and  to 
the  indications  of  implication  of  the  medulla.     Bulbar  symptoms 


CHRONIC  MUSCULAR  ATROPHY.  557 

increase  the  gravity  of  tlie  prognosis,  especially  when  definite  weak- 
ness can  be  recognised.  Slight  vague  difl&culty  in  articulation  may 
remain  stationary  for  years,  and  does  not  necessarily  render  the 
prognosis  worse,  especially  if  the  atrophy  elsewhere  does  not  increase 
rapidly.  If  the  malady  ceases  to  advance,  the  prospect  of  any  recovery 
depends  on  the  rate  at  which  the  disease  has  progressed.  Recent 
rapid  loss  of  power  may  be  to  some  extent  recovered  from,  especially 
when  the  muscles  present  the  degenerative  reaction.  Wasting  that 
has  existed  for  six  months  will  probably  persist  unchanged.  In  a 
typical  chronic  case  there  is  little  hope  of  any  actual  recovery  of  tissue 
or  power.  The  effects  depend  on  a  slow  destruction  of  nerve-elements, 
the  renewal  of  which  seems  to  be  impossible. 

Treatment. — The  first  important  element  is  to  secure  favorable  con- 
ditions of  life,  and  to  maintain  the  general  health  in  as  perfect  a  state 
as  possible.  Fresh  air  and  gentle  exercise  are  important,  but  all 
fatiguing  exertion  should  be  avoided,  and  likewise  all  mental  strain. 
When  the  patient  becomes  helpless,  great  care  is  necessary.  Bedsores 
in  this  disease  mean  inattention,  and  may  always  be  avoided. 

Only  one  method  of  treatment  has,  in  my  own  experience,  shown 
itself  capable  of  arresting  the  disease,  not  indeed  in  aU,  but  in  more 
than  half  the  cases  in  which  it  has  been  employed.*  It  is  the  admin- 
istration of  strychnia  by  hypodermic  injection.  In  seven  almost  con- 
secutive cases,  in  middle  life,  this  treatment  has  been  followed  by 
arrest  within  a  month  of  its  commencement,  and  the  arrest  has  been 
permanent  in  all  the  cases  but  one.  In  the  senile  cases  the  treatment 
has  failed,  but  in  most  of  them  the  disease  was  in  an  advanced  stage, 
and  the  lumbar  cord  had  begun  to  suffer.  In  some  of  the  cases 
in  which  the  result  was  prompt  and  distinct,  strychnia  given  by  the 
mouth  had  failed.  It  is  conceivable  that  the  different  result  is  due 
to  the  fact  that  the  agent  is  brought  into  more  rapid  contact  with  the 
nerve-elements,  perhaps  in  purer  form.  Its  action  may  possess  greater 
momentum,  as  it  were,  and  may  thus  exert  an  influence  on  the  nutrition 
of  the  nerve-elements  much  more  considerable  than  when  it  is  slowly 
absorbed  from  the  alimentary  canal.  One  injection  daily  has  been 
given,  at  any  convenient  place.  The  nitrate  is  the  most  convenient 
salt,  one  fiftieth  of  a  grain  at  first,  quickly  increased  to  one  twentieth 
or  one  fifteenth.  The  injections  need  to  be  continued  for  months. 
When  the  malady  is  apparently  arrested,  it  is  well  to  intermit  the 
injections  for  one  week  in  three  or  four.  Other  nervine  tonics  seldom 
exert  a  distinct  influence,  but  those  that  are  useful  in  other  degenera- 
tive diseases,  as  tabes,  such  as  arsenic,  may  be  given  by  the  mouth  at 
the  same  time  as  the  injections  are  employed.      In  a  malady  so  grave 

*  At  first  I  regarded  the  apparent  result  with  doubt,  but  careful  and  repeated 
observations  have  made  it  impossible  to  consider  the  arrest  of  the  disease  as  other 
than  the  direct  effect  of  the  treatment.  It  may  be  asked.  Why  should  not  a  larger 
dose  by  the  mouth  be  equally  effective  P  It  does  not  seem  to  be,  is  the  only  answer 
that  can  be  given. 


558  SPINAL    CORD. 

it  is  desirable  to  neglect  nothing  that  may  possibly  exert  a  beneficial 
influence. 

Local  treatment  of  the  muscles  has  very  little  influence  on  the 
wasting,  as  may,  indeed,  be  expected  from  its  nature.  The  most 
sedulous  and  skilful  use  of  electricity,  voltaic  or  faradic,  fails,  as  a 
rule,  to  produce  alone  any  effect  on  the  course  of  the  disease.  If  the 
malady  is  progressing  at  the  same  rate  in  each  arm,  and  the  muscles  of 
one  arm  are  regularly  treated  with  electricity,  while  those  in  the  other 
arm  are  left  alone,  no  difference  can  be  detected  in  the  rate  of  wasting 
on  the  two  sides.  It  is  possible,  nevertheless,  that  electricity  some- 
times does  a  little  good.  In  cases  in  which  a  rapid  loss  of  power  has 
occurred,  and  weakness  is  out  of  proportion  to  the  wasting,  some  re- 
covery is  possible,  and  there  is  no  doubt  that  the  excitability  of  the 
muscular  tissue  is  maintained  for  a  longer  time  by  galvanism,  although 
the  bulk  of  the  muscle  may  not  be  influenced.  In  other  cases  all  that 
can  be  said  is  that  the  influence  of  electricity,  properly  applied,  is  in 
the  right  direction.  Moreover  the  disease  is  one  of  those  in  which 
patients  find  it  hard  to  believe  that  electricity  cannot  help  them,  and 
the  probability  is  that  their  conviction  will  be  fostered  by  some  medical 
adviser.  It  is  one  of  the  diseases  in  which  unjustifiable  assertions  are 
too  often  made  that  early  electrical  treatment  would  have  been  suc- 
cessful. It  may  be  weU,  therefore,  if  only  to  satisfy  the  patient  that 
nothing  has  been  left  untried,  that  a  careful  course  of  electrical 
treatment  should  be  adopted.  Faradism  may  be  used  if  the  muscles 
are  sensitive  to  it,  but  i£  they  present  any  greater  irritability  to 
voltaism  it  is  better  to  use  this.  It  is  immaterial  whether  the  appli- 
cation be  confined  to  the  muscles  or  whether  one  electrode  be  placed 
over  the  affected  part  of  the  spinal  cord.  The  latter  method  has  no 
disadvantages,  but  my  own  observations  have  failed  to  confirm  the 
confident  statements  sometimes  made  regarding  its  superiority.  It  is 
very  important  that  the  current  strength  employed  should  be 
moderate.  Strong  applications  often  cause  much  subsequent  paia, 
and  even  increased  disability,  and  should  be  carefully  avoided.  I 
have  known  a  rapid  increase  of  weakness  follow  a  strong  application 
in  a  way  that  convinced  the  patient,  at  least,  that  the  two  were 
connected. 

Rubbing  and  massage  of  the  muscles  have  been  frequently  employed, 
and  of  this  treatment  also  it  may  be  said  that  its  influence  is  in  the 
rio-ht  direction,  although  usually  inappreciable  so  far  as  the  muscular 
atrophy  is  concerned.  Combined  with  passive  movement,  the  influence 
of  rubbing  in  preventing  and  diminishing  deformities  is  more  distinct. 
No  special  bath  treatment  is  of  service.  When  the  disease  occurs  in 
the  subjects  of  syphilis,  specific  treatment  invariably  fails,  and  I  have 
even  known  the  progress  of  the  disease  to  be  distinctly  accelerated  by 
an  energetic  course  of  treatment  both  with  iodide  and  with  mercury. 
It  is  important  that  all  treatment  should  be  pursued  in  moderation, 
and  that  its  effects  shoxdd  be  carefully  watched. 


AETHEITIO   MUSCOLAE   ATEOPHT.  659 


Aetheitic  Muscttlae  Ateopht. 

Articular  inflammation  is  almost  invariably  attended  "witli  rapid 
wasting  of  the  muscles  that  move  the  joint.  This  occurs  equally, 
whatever  be  the  cause  of  the  inflammation,  and  whether  this  is 
spontaneous  or  traumatic.  It  attends  chronic  as  well  as  acute  inflam- 
mations, and  occurs  in  animals  if  joint  inflammation  is  produced  in 
them.* 

The  muscles  that  waste  are  chiefly  those  which  extend  the  affected 
joint.  The  atrophy  is  well  seen  in  the  muscles  in  front  of  the  thigh 
when  the  knee  is  inflamed.  If  the  ankle  is  affected,  the  calf  muscles 
chiefly  waste ;  if  the  hip,  the  glutei ;  if  the  wrist,  the  extensor  muscles  of 
the  forearm ;  the  triceps  when  the  elbow  is  affected ;  the  deltoid  when 
the  shoulder- joint  is  inflamed.  In  rheumatoid  arthritis  of  the  finger- 
joints  such  wasting  is  usually  very  conspicuous  in  the  interossei,  and 
especially  in  the  abductor  indicis.  The  atrophy,  however,  sometimes 
involves  the  flexors  as  well  as  the  extensors,  and  rarely  muscles  of  the 
limb  that  are  near  but  do  not  move  the  affected  joint.  In  very  rare 
cases  all  the  muscles  of  a  limb  have  presented  some  wasting.  Cases 
in  which  the  atrophy  is  unusual  in  distribution,  and  especially  when 
it  is  on  the  distal  side  of  the  affected  joint,  must  be  regarded  with 
some  suspicion,  because  inflammation  may  have  spread  from  the  joint 
to  a  nerve,  and  distant  wasting  may  have  been  thus  produced.  It  is 
said  that  there  is  sometimes,  at  the  onset,  a  considerable  weakening 
of  the  muscle,  interfering  with  movement  more  than  can  be  accounted 
for  by  the  pain  in  the  joint  which  movement  causes,  and  that  such 
initial  palsy  is  transient  (Yaltat,  Duchenne).  But  the  pain,  by  its 
inhibitory  influence,  usually  obscures  such  palsy,  which  is  indeed  con- 
fessedly rare. 

The  atrophy,  on  the  other  hand,  may  be  regarded  as  almost  con- 
stant. If  the  onset  of  the  joint  affection  is  acute,  the  wasting  occurs 
rapidly;  in  a  week  or  ten  days  a  difference  in  the  circumference  of 
the  limb  may  be  detected  by  measurement.  However  long  the  affected 
muscle  may  be,  the  wasting  involves  the  whole  length  of  it,  and  not 
merely  the  part  in  the  neighbourhood  of  the  diseased  joint.  The 
degree  varies ;  usually  moderate,  and  sometimes  slight,  it  is  occa- 
sionally considerable,  so  that  the  femur  may  be  readily  felt  when  the 
quadriceps  femoris  is  the  seat  of  the  wasting ;  or  the  head  of  the 
humerus  may  be  distinct,  and  the  acromion  prominent,  if  the  deltoid 
is  affected.  Although  the  whole  length  of  the  affected  muscle  is 
always  involved,  if  the  muscle  is  a  wide  and  compound  one  some 
parts  may  suffer  chiefly.     Thus  in  the  thigh  all  parts  of  the  extensor 

•  Valtafc, 'Archives  generales,*  1877,  tome  xxx.  pp.  159  and  321.  The  subject 
has  been  discussed  by  Vulpian  (•Le9ons  sur  I'App.  Vaso-moteur,'  1875,  t.  ii)  and  by 
Paget  ('  Lancet,*  1873,  vol.  ii,  p.  727,  in  a  lecture  republished  in  •  Clinical  Lectures 
and  Essa^  s,'  1875,  p.  208). 


560  SPINAL    COED. 

may  waste  equally,  or  the  rectus  or  vastus  internus  may  suffer  more 
tlian  the  other  parts.  Tho  wasting  increases  during  two  or  three 
weeks,  then  becomes  stationary,  but  continues  as  long  as  the  joint 
disease  lasts.  When  the  joint  has  recovered,  the  muscles  in  most 
3ases  slowly  regain  their  normal  size.  Occasionally,  especially  when 
the  arthritis  has  lasted  a  long  time,  the  wasting  may  continue  for 
months  or  years  after  the  joint  disease  is  at  an  end. 

The  electrical  irritability  of  the  atrophied  muscles  may  be  normal, 
but  is  often  slightly  lowered,  equally  to  faradism  and  voltaism.  The 
change  is  trifling,  to  be  recognised  only  by  comparison  with  the  other 
side.  There  is  generally  a  distinct  and  sometimes  a  considerable 
local  increase  in  myotatic  irritability;  the  knee-jerk  is  excessive  if 
the  thigh  muscles  are  affected,  and  a  rectus- clonus  can  sometimes  be 
obtained.  A  foot-clonus  may  be  elicited  when  the  ankle-joint  is 
affected.  Occasionally  the  increased  irritability  extends  beyond  the 
region  of  atrophy,  so  that,  for  instance,  a  foot-clonus  may  be  obtained 
when  the  knee-joint  and  thigh  muscles  are  affected,  although  there  is 
no  wasting  below  the  knee.  As  an  example  of  this,  which  illustrates 
also  the  occasional  persistence  of  the  atrophy,  may  be  mentioned  the 
case  of  a  young  man  who  jumped  over  a  hoarding  and  twisted  his  left 
leg  in  doing  so.  He  felt  immediately  severe  pain  in  the  knee,  the 
joint  quickly  swelled,  and  became  the  seat  of  an  acute  inflammation 
which  lasted  for  several  weeks,  and  then  slowly  subsided.  During  the 
inflammation  the  thigh  wasted.  I  saw  him  two  years  after  the  onset, 
and  there  was  still  considerable  wasting,  involving  the  whole  of  the 
extensor  of  the  knee.  The  minimum  circumference  of  the  left  thigh 
above  the  knee  was  three  quarters  of  an  inch  less  than  that  of  the 
right,  although  there  was  no  difference  between  the  two  legs  below  the 
knee.  There  was  a  slight  diminution  in  f aradic  and  voltaic  irritability 
in  the  affected  muscles.  The  knee-jerk  was  much  more  considerable 
on  the  left  than  on  the  right  side,  and  there  was  well-marked  foot- 
clonus  in  the  left  leg,  but  none  in  the  right.  A  year  and  a  half  later 
the  symptoms  were  unchanged.  Very  rarely  some  contracture  occurs 
in  the  opponents  of  the  atrophied  muscles.  Sensory  symptoms  are  as 
a  rule  absent,  but  there  may  be  slight  tingling  in  the  skin  during  the 
acute  stage  ;  it  is  said  that  areas  of  anaesthesia  sometimes  develop,  but 
probably  in  such  cases,  inflammation  has  extended  to  a  nerve  iu  the 
vicinity  of  the  joiat. 

Pathologt. — Few  facts  have  been  ascertained  regarding  the  con- 
dition in  man.  In  one  case,  carefully  investigated,  the  only  change 
was  iu  the  muscles,  and  consisted  merely  in  a  narrowing  of  the  fibres, 
one  half  of  which  were  below  the  normal  average,  while  no  less  than 
84  per  cent,  fell  short  of  a  standard  which,  in  health,  only  21  per  cent, 
failed  to  reach.*  A  few  presented  longitudinal  striation  (as  in 
Fig.  150),  and  some  of  noi-mal  width  were  unduly  tortuous;  other- 
wise their  aspect  was  normal.  The  sheath  nuclei  were  proliferated  in 
*  Darkschewitsch, '  Neur.  Cent.,'  1891,  p.  353. 


AETHRITIC    MUSCULAR  ATEOPHT.  •  561 

places,  and  apart  from  this  change  tlie  intermediate  substance  pre- 
sented local  increase  in  quaatity,  especially  where  the  fibres  were 
narrower  than  normal.  This  observation  agrees  with  those  that  have 
been  made  on  animals,  in  which  the  condition  has  been  produced,  and 
in  them,  moreover,  the  motor  nerves  have  been  fotmd  normal 
(Yaltat).*  Indications  of  inflammation  have  been  fonnd  in  the  joint 
nerves.t  as  might,  indeed,  be  expected,  whatever  the  mechanism  of  the 
atrophy.  Visible  changes  in  the  spinal  cord  have  never  been  discovered, 
but  this  does  not  exclude  nutritional  changes. 

We  may,  therefore,  exclude  from  the  possible  causes,  all  forms  of 
motor  neuritis,  both  simultaneous,  due  to  the  cause  of  the  joint 
inflammation,  and  consecutive,  communicated  to  the  adjacent  nerve 
from  the  joint.  The  latter  (a  secondary  extension  of  inflammation 
to  the  nerve)  would  manifestly  not  explain  the  affection  of  the 
whole  of  a  muscle  on  the  proximal  side  of  the  affected  joint — as, 
for  instance,  atrophy  of  the  whole  extensor  in  arthritis  of  the  knee — 
although  the  extension  does  occur,  and  must  be  recognised  as  an 
occasional  event. ij:  We  must  also  recognise  the  possibility  of  a  simul- 
taneous neuritis,  with  acute  changes  in  the  nerve-fibres,  manifested 
by  the  reaction  of  degeneration  in  the  muscles.  The  event  is,  how- 
ever, exceptional.  It  is  certainly  separable  and  to  be  separated  from 
the  common  arthritic  atrophy ;  but  it  is  not  easy  to  keep  apart  the 
two  varieties  of  neuritis — the  simultaneous  and  the  consecutive — 
when  a  case  is  seen  only  long  after  the  onset,  because  a  neuritis  that 
arises  by  extension  may  travel  along  the  nerve  and  present  an  ulti- 
mate distribution  quite  different  from  that  which  it  possessed  at  the 
onset. 

For  ordinary  arthritic  atrophies  we  must  seek  some  other  explana- 
tion. The  nature  of  their  cause  long  ago  suggested  to  Paget  a  "  reflex 
influence "  on  the  muscles  as  the  probable  mechanism,  and  Yulpian 
and  Charcot  have  suggested  a  similar  hypothesis,  assuming  a  derange- 
ment of  the  nutrition  and  influence  of  the  motor  cells  of  the  cord,  to 
be  determined  by  the  morbid  impulses  from  the  joint  nerves,  and  to 
determine  the  alterations  in  the  muscles.  The  theory  receives  impor- 
tant support  from  the  fact  that  the  wasting  of  the  muscles  is  pre- 
vented by  previous  division  of  the  posterior  spinal  roots  (Eaymond), 
which  amounts,  indeed,  to  an  indirect  demonstration  of  the  fact  that 
%  reflex  process  §  underlies  the  phenomena.  It'  is  doubtful  whether 
we  can  go  much  further  than  this.  By  preceding  hemisection  of  the 
cord  it  is  increased  on  the  side  of  the  operation ;  but  the  conditions 

•  Valtat,  'De  I'Atropliie  Muse,  &c.,'  Paris,  1877.  See  also  Deroche,  *]Stude 
cliu.  et  exp.,'  Paris,  1890. 

t  See  Duplay  and  Cazin,  'Arch,  gen.,'  January,  1891. 

X  E.g.  to  the  ulnar,  in  rheumatoid  arthritis  of  the  hand  (Bury,  *Med.  Chron.,* 
1888,  p.  182).  See  on  this  subject  A.  E.  Garrod,  *Med.-Chir.  Trans.,' Ixxi,  265 ; 
also  Pitres  and  Vaillard, '  Rev.  de  Med.,'  1887,  No.  6. 

§  R:iymond,  *  Kev.  de  Med.,'  1890,  374. 

VOL.  I.  36 


562  SPINAL   COED. 

are  here  more  complex,  and  tlie  demonstration  of  tlie  reflex  relation 
less  simple,  than  in  the  result  of  division  of  the  roots. 

The  increased  knee-jerk,  &c.,  that  attend  the  wasting,  and  the 
curious  fact  that  arthritis  may  set  up  a  degeneration  of  the  spinal 
cord,  apparently  beginning  in  the  termination  of  the  pyramidal  fibres 
(see  p.  493),  should  be  kept  in  mind.  They  are  certainly  significant, 
and  suggest  that  the  influence  from  the  joint  nerves  acts  on  the  "  con- 
trolhng  structure  "  of  the  muscle-reflex  centre  (see  p.  240)  ;  but  more 
facts  are  needed  before  a  valid  hypothesis  can  be  framed. 

Diagnosis. — The  moderate  degree  of  the  wasting,  with  correspond- 
ing change  in  electrical  reactions,  coupled  with  the  wide  extent  of 
the  alteration,  embracing  the  whole  of  the  muscles  involved,  constitute 
a  distinctive  characteristic — distinctive,  at  least,  when  taken  in  con- 
nection with  the  preceding  joint  affection  which  is  the  cause  of  the 
atrophy.  A  primary  neuritis,  causing  secondary  arthritic  adhesions, 
has  its  own  features — ^their  wider  range,  so  far  as  function  is  concerned, 
and  especially  their  limitation  to  nerve  distribution.  Hardly  any  other 
malady  is  likely  to  be  confused  with  it,  except  some  graver  disease, 
while  still  in  an  early  sta'--^ ;  but  such  an  affection  can  only  be  dis- 
tinguished by  waiting  until  the  nature  of  the  malady  has  had  time  to 
show  itself. 

Peognosis. — When  the  inflammation  of  the  joint  is  brief  in  dura- 
tion, recovery  of  the  muscles  may  be  anticipated  with  confidence. 
In  children,  even  after  prolonged  joint  disease,  the  muscles  usually 
regain  their  normal  bulk.  In  adults  the  wasting  often  lasts  for  a  long 
time  after  the  joint  is  well,  and  a  cautious  prognosis  shoidd  be  given 
if  the  arthritis  has  lasted  long.  Even  if  sHght  wasting  is  persistent, 
normal  power  is  usually  recovered ;  but,  as  the  case  mentioned  above 
shows,  the  symptoms  sometimes  continue  for  years.  The  prognosis 
should  be  especially  cautious  where  there  is  a  marked  increase  in 
myotatic  irritability,  or  if  the  atrophy  shows  a  disposition  to  involve 
other  muscles  of  the  limb  than  those  concerned  in  moving  the  affected 
joint. 

Treatment. — The  chief  treatment  is  local, — electrical  stimulation 
of  the  muscles,  and  gentle  rubbing.  It  is  doubtful  whether  drugs 
have  any  influence  on  the  condition,  but  small  doses  of  strychnia  may 
be  given,  or  added  to  whatever  agent  is  given  for  the  joint  affection. 
As  long  as  the  joint  is  inflamed,  treatment  rarely  causes  any  increase 
in  the  bulk  of  the  muscle.  It  is  of  great  importance  to  secure  the 
early  recovery  of  the  joint,  and  to  avoid  aU  influences  that  cause  pain 
in  it,  since,  as  we  have  seen,  it  is  apparently  through  the  sensory 
nerves  that  the  joint  affection  exerts  its  injurious  influence.  When 
the  arthritis  has  ended,  the  muscles  usually  recover  slowly  without 
assistance,  but  it  is  probable  that  the  local  treatment  accelerates  the 
process.  The  form  of  electricity  is  of  little  consequence;  either 
faradism  or  voltaism  may  be  employed,  but  snould  only  be  used 
in  suf&cient  strength  to  cause  gentle  contraction.    The  muscles  act 


MUSCULAR  DYSTROPHIES.  563 

readily  to  faradism,  and  a  mild  faradic  current  is,  as  a  rule,  tlie  best 
to  employ.  All  painful  stimulation  of  the  sensory  nerves  should  be 
avoided. 

MUSCTJLAB  AtEOPHY   PROM   OvEE-r*!!!. 

Muscles  that  are  much  used  sometimes  waste.  The  effect  is 
seen  most  frequently  in  the  small  muscles  of  the  hand,  especially 
in  persons  of  weakly  constitution,  who  use  these  muscles  unduly. 
Thus  a  young  lady  devoted  many  hoiurs  a  day  to  "  illuminating," 
during  several  years,  and  then  some  muscles  of  the  thenar  emiuenco 
began  to  waste,  and  became  considerably  atrophied.  Such  wasting 
is  scarcely  ever  met  with  in  the  larger  muscles,  but  has  been  observed 
in  the  biceps,  consecutive  to  hypertrophy,  in  Sheffield  smiths 
(Frank- Smith;.  The  electric  irritability  is  gradually  lowered  in  this 
local  wasting,  equally  to  both  currents,  as  it  is  in  progressive  muscular 
atrophy.  We  do  not  know  whether  this  wasting  from  over- use  is 
purely  local,  or  whether  the  related  ganglion-cells  of  the  spinal  cord 
fail  first,  and  the  muscular  wasting  is  secondary  to  their  atrophy. 
Gull  said  many  years  ago,  "  it  is  as  reasonable  to  infer  a  lesion  of  the 
grey  matter  from  overwork  as  of  the  muscles."*  The  wasting  often 
persists  when  the  over-use  of  the  muscles  is  discontinued.  Hyper- 
trophy from  over-use  does  not  usually  give  place  to  atrophy.  These 
two  facts  are  somewhat  in  favour  of  the  view  that  the  lesion  is 
primarily  of  the  nerve-cells.  The  wasting  in  these  cases  shows  no 
tendency  to  extension  beyond  its  original  limits.  The  treatment 
is,  first,  the  cessation  from  the  excessive  exertion  of  the  muscles; 
secondly,  the  improvement  of  the  general  health,  and  the  adminis- 
tration of  nerve  tonics,  especially  of  strychnine ;  and  thirdly,  the 
gentle  electrical  stimulation  of  the  muscles  by  either  faradism  or 
voltaism. 


THE   MUSCULAR   DTSTROPHIES. 

Idiopathic  atrophy,  which  is  primarily  muscular,  although  it  is  not 

one  of  the  diseases  of  the  nervous  system,  is  commonly  described  with 
them,  because  it  frequently  presents  so  close  a  resemblance  to  the 
spinal  atrophy  just  described  as  to  be  distinguished  with  some  diffi- 
culty. Moreover,  even  the  varieties  that  do  not  present  this  close 
resemblance  were  for  a  time,  and  indeed  until  lately,  believed  to  be  of 
central  nature,  to  depend  on  the  spinal  cord,  and  to  be  rightly  included 
among  its  diseases.  Hence  the  custom  of  thus  describing  them  has 
become  established,  and  has  now  to  be  followed,  although  we  know 
that  it  is  based  on  a  mistaken  theory.    At  the  same  time,  not  only 

•  '  Guy's  Hosp.  Reports,'  1862,  p.  246. 


b(^i  SPINAL   CORD. 

has  tlie  raethod  the  advantage  of  convenience,  but  it  has  also  some 
measure  of  scientific  justification.  The  muscles  are  more  closely 
allied  to  the  nerves  than  to  any  other  structures,  as  regards  both 
physiological  properties  and  pathological  susceptibility,  and  it  ia 
doubtful  whether  any  other  association  would  better  befit  these 
diseases. 

The  idiopathic  myopathies  have  received  much  attention  during 
the  last  few  years,  and  many  new  facts  have  been  ascertained  with 
regard  to  the  different  varieties  and  their  relations  to  each  other.  The 
treatise  by  Erb*  has  had  the  effect  of  giving  unity  and  cohesion  to 
w^hat  was  formerly  a  collection  of  disconnected  types,  and  has  con- 
clusively demonstrated  the  essential  identity  of  the  different  varieties 
hitherto  described.  The  so-called  pseudo-hypertrophic  variety  of 
muscular  affection  has  been  recognised  under  this  name  ever  since  its 
graphic  description  by  Duchenne.f  The  features  of  this  were  muscular 
weakaess  associated  with  enlargement  of  muscles,  sometimes  a  few, 
sometimes  many.  He  also  described  in  the  same  work  'I  another  form 
of  paralysis  which  he  supposed  to  be  similar  in  its  etiology  to  the 
progressive  muscular  atrophy  of  adults,  and  which  he  named  "  atrophic 
musculaire  progressive  de  I'enfance."  This  was  characterised  by 
wasting  of  the  muscles  of  the  face  as  well  as  of  the  limbs,  and  also  by 
its  tendency  to  affect  more  than  one  member  of  a  family.  Leyden  in 
1875  §  proposed  to  separate  the  hereditary  forms  of  muscular  atrophy 
from  those  of  the  Aran-Duchenne  type.  He  pointed  out  that  the 
hereditary  form  tends  to  commence  in  early  life,  to  affect  several 
members  of  the  same  family,  especially  the  males,  and  he  directed 
attention  to  the  resemblance  between  the  hereditary  form  and  the 
pseudo-hypertrophic  of  Duchenne.  In  1879  ||  Gowers  in  a  clinical 
lecture  gave  a  full  description,  from  numerous  clinical  observations,  of 
pseudo-hy3)ertrophic  paralysis,  and  also  set  out  all  that  was  known  of 
its  pathological  anatomy,  and  in  this  lecture  clearly  recognised  that 
hypertrophy  and  atrophy  may  be  combined  in  different  proportions, 
and  that  there  are  cases  that  connect  the  two  extremes — in  some 
enlargement  of  many  muscles,  in  others  wasting  in  all.  Such  a  view 
affords  a  glimpse  of  the  group  which  Erb  ^  afterwards  clearly  outlined 
under  the  name  of  the  "  juvenile  form  of  progressive  muscular 
atrophy." 

Prom  a  consideration  of  the  characters  and  histories  of  these  cases 
Erb  concluded  that  the  three  varieties — ^the  juvenile  form,  the  here- 
ditary form  of  Leyden,  and  pseudo-hypertrophic  paralysis — were  all 
varieties  of  one  condition,  for  which  he  proposed  the  designation 

*  *  Dystrophia  mascnlaris  progressiva,'  Leipzig,  1891. 

+  '  Electrisation  localis^,'  3rd  edit.,  p.  595. 

X  Ibid.,  p.  518. 

§  '  Klinik  der  Ruck.  Krank.,*  ii,  p.  525. 

||  '  Lancet,'  1879. 

%  «  Deutsch.  Arch.  f.  klin.  Med./  Bd.  xxxiv,  8.  467. 


MUSCULAE   DYSTfiOPHIES.  565 

*'  dystrophia  muscularis  progressiva."  The  next  important  step  in  tlie 
development  of  our  knowledge  took  j)lace  in  18P5,  when  Landouzy 
and  Dejerine  published*  an  exhaustive  paper  on  what  they  named 
"  Myopathic  Atrophique."  It  dealt  with  observations  of  an  exceed- 
ingly intevest:jig  group,  the  essential  characteristics  of  which  were 
wide-spread  muscular  atrophy  commencing  in  the  face,  and  without 
hypertrophy.  These  cases  were  really  identical  with  tho-e  described 
by  Duchenne  as  "atrophie  musculaire  progressive  de  I'enfance,"  but 
the  later  observers  completed  the  description  by  the  discovery  from 
post-mortem  examination  that  it  did  not  depend  upon,  nor  was  it 
associated  with,  changes  in  the  spinal  cord,  but  that  it  was  a  muscular 
affection  without  detectable  nervous  lesions.  They  regarded  their  cases 
as  constituting  a  distinct  and  separate  variety  of  myopathy,  relying 
upon  the  commencement  in  the  face  and  the  absence  of  hypertrophy, 
and  they  refused  to  recognise  the  essential  identity  of  their  cases  with 
those  of  Erb,  in  spite  of  the  fact  that  in  one  of  their  quoted  cases  the 
face  was  not  affected,  and  considerable  enlargement  of  the  calves,  if  it 
was  not  present  at  the  time,  had  been  a  few  years  before. 

The  latest  and  most  important  contribution  to  the  elucidation  of 
this   disease  is   the  work  of  Erb  already  alluded  to.f      In  this  he 
subjects  to  close  examination  numerous  clinical  records  of  cases  of 
the  different  varieties  already  referred  to,  and  makes  it  clear  by  the 
observation  of  cases  of  different  types  among  members  of  the  same 
family,  by  the  description  of  cases  which  form  distinct  connecting  links 
between  the  different  varieties,  and  by  the  observation  of  the  histo- 
logical conditions  in  the  affected  muscles,  that  all  four  varieties — 
pseudo-hypertrophic  paralysis,  the  juvenile  form  of  Erb,  the  Lan- 
douzY-Dejerine  type, — identical  with   the   atrophie   musculaire  pro- 
gressive de  I'enfance  of  Duchenne — are  but  branches  of  a  parent  stem, 
and  essentially  the  same  disease.     He  proposes  a  division  into  two 
main   groups,  viz.    (1)  children,  (2)  adults ;    and  in  the  former  he 
would  distinguish  two  varieties,  the  hypertrophic  and  the  atrophic. 
In  the  hypertrophic  he  would  further  distinguish  those  with  pseudo- 
hypertrophy and  those  with  true  hypertrophy,  and  in  the  atrophic 
variety  those  with,  and  those  without  involvement  of  the  face.    Such  a 
division  is  of  course  an  arbitrary  one,  for  even  between  the  two  chief 
varieties  no  hard  and  fast  line  can  be  drawn  ;  and  the  same  is  true  in  a 
greater  degree  of  the  minor  divisions.      It  has,  therefore,  seemed  best 
to  describe  the  disease  on  the  same  lines  as  have  been  followed  in 
previous  editions  of  this  book,  with  a  due  recognition  of  the  fact  that 
any  particular  case  met  with  may  partake  of  the  characters  of  simple 
atrophy  and  also  of  pseudo-hypertrophic  atrophy,  and  indeed  may  at 
one  time  be  regarded  as  belonging  to  one  group,  and  at  another  to  the 
other. 

All  the  diseases  of  this  class  seem  to  depend  on  a  defective  tendency  in 

*  '  Rev.  de  Medecine,'  1855. 

f  •  Dystrophia  musnularis  progressiva,'  Leipzig,  1891. 


566  SPINAL    COED. 

tlie  development  of  tlie  germinal  tissue  wMcli  forms  muscles ;  tliej  are 
essentially  congenital  diseases.  Although  they  are,  in  most  cases, 
merely  potential  maladies  at  the  time  of  birth,  and  sometimes  for 
years  afterwards — even  occasionally  during  a  considerable  part  of 
life, — the  morbid  tendency  does  sometimes  attain  actual  development 
in  the  earlier  years.  But  we  know  nothing,  or  almost  nothing,  at 
present,  of  any  other  cause  than  this  developmental  tendency;  we 
know  nothing  of  these  diseases  as  acquired  maladies,  the  result  of 
influences  acting  on  a  healthy  and  normal  organism. 

Another  fact  of  their  general  pathology  is  analogous  to  that  which 
we  have  already  considered  in  connection  with  developmental  diseases 
of  the  central  nervous  system.  It  depends  on  the  double  constitution 
of  muscular  as  of  nervous  organs,  on  the  presence  of  interstitial  con- 
nective tissue  between  the  contractile  elements,  and  on  the  relation  of 
both  to  trophic  tendencies.  An  overgrowth  of  the  interstitial  tissue 
may  concur  with  wasting  of  the  fibres,  and  this  overgrowth  may  be 
abnormal  in  its  features.  Hence  Erb  has  chosen  the  term,  abeady 
referred  to,  "  muscular  dystrophy,"  as  a  more  precise  designation  for 
the  class — a  term  which  is  likely  to  obtain  general  acceptance.  The 
important  fact  to  recognise  is  that  there  may  be  either  a  simple  defect 
in  the  growth  of  the  fibres,  ultimately  leading  to  their  disappearance, 
or  there  may  be,  with  this,  an  overgrowth  of  the  connective  tissue, 
either  a  simple  hyperplasia,  or  a  perverted  growthin  which  f  at-ceUs  form. 
These  cells  increase  its  bulk,  but  are  ultimately  removed.  The  muscular 
fibres  also  sometimes  present  hypertrophic  enlargement,  but  simple 
wasting  is  the  most  common  change. 

The  symptoms  by  which  these  changes  are  manifested  vary  accord- 
ing to  the  nature  and  seat  of  the  alteration.  In  all  cases  muscular 
power  fails  as  the  contractile  fibres  waste.  The  chief  difference  in 
aspect,  however,  depends  on  the  seat  of  the  affection  and  the  effect  of 
the  interstitial  changes,  and  especially  on  the  frequent  enlargement  of 
muscles  from  the  formation  of  fat  in  the  fibrous  tissue.  This  produces 
the  semblance  of  hypertrophy  that  has  led  to  the  name  "pseudo- 
hypertrophic -paralysis  "  being  applied  to  the  cases  in  which  it  is 
present.  The  amount  of  enlargement  varies  much  ]  the  fibrous  tissue 
alone  may  cause  none,  but  it  is  sometimes  great  and  wide-spread  in 
the  cases  in  which  fat  is  formed.  In  all,  however,  a  primary  shrinking 
of  the  muscles,  often  preceded  by  a  true  enlargement  of  the  fibres,  is 
due  to  the  wasting  of  their  proper  substance,  the  result  mainly  of  a 
defective  tendency  of  vital  endurance  or  growth,  and  only  in  a  slight 
degree  to  the  influence  of  the  interstitial  tissue  when  this  is  increased 
in  quantity. 

Thus  certain  varieties  are  constituted  by  the  external  manifestation 
of  the  muscular  changes.  Even  in  the  class  in  which  pseudo-hyper- 
trophy occurs  there  may  be  no  change,  or  only  diminution  in  the  size 
of  the  muscles,  but  with  increased  firmness,  as  the  accompaniment 
of  the  lessened  power.      When  there  is  enlargement,  this  may  or  may 


PSEUDO-HYPERTROl'HIC   MUSCULAR    PARALYSIS.  567 

not  correspond  in  time  to  the  loss  of  power,  since  the  mnscnlar  fibres 
may  waste  cither  during  interstitial  growth,  or  after  this  tissue  has 
undergone  the  atrophy  which  ultimately  supervenes.  Thus  we  may 
have  two  classes  of  cases  belonging  to  this  type,  one  with  enlargement 
of  muscles,  few  or  many,  the  other  with  only  wasting  of  muscles,  the 
two  corresponding  in  position  and  in  the  general  course  and  relations 
of  the  affection. 

In  another  form  there  is  never  an  interstitial  growth  of  fat,  and 
probably  no  considerable  increase  of  fibrous  tissue ;  simple  atrophy 
of  the  fibres  is  its  pathological  characteristic,  while  primary  shrinking 
of  the  muscles  is  its  external  manifestation.  The  shoulder  muscles  are 
prone  to  suffer  most,  and  in  some  cases  there  is  a  peculiar  affection  of 
the  face.  These  cases  are  also  more  variable  in  the  time  of  life  at 
which  the  symptoms  begin.  But  these  varieties,  as  we  have  already 
state  i,  are  connected  by  cases  which  to  some  extent  combine  the 
various  features.  Such  combined  forms  forbid  the  separation  of  the 
types,  which,  nevertheless,  frequently  maintain  so  distinct  a  coui'se 
in  many  members  of  a  family,  even  through  several  generations, 
that  we  are  compelled  to  recognise  their  distinctness,  although  we 
cannot  divide  them  altogether. 

The  essential  element  in  the  disease  has  been  spoken  of  as  a  "  quali- 
tative" defect,  which  entails  an  imperfect  development,  manifested 
sooner  or  later  by  the  defective  vitality  of  the  proper  elements  of  the 
tissue.  Bat  there  is  reason  to  believe  that  the  defect  is  not  always 
merely  qiia,litative.  In  some  cases  the  defect  in  certain  muscles  is  so 
absolute  at  so  early  a  period  as  to  make  it  almost  certain  that  these 
muscles  or  parts  of  muscles  are  congenitally  absent,  and  that  the 
germinal  effect  is  thus  quantitative  as  well  as  qualitative.  The 
muscles  in  which  this  apparent  failure  is  observed  vary  in  the  several 
forms,  and  will  be  mentioned  in  the  account  of  these. 

The  peculiar  form  of  atrophy,  which  differs  from  the  others  in  the 
early  affection  of  the  muscles  supplied  by  the  peroneal  nerve,  is 
separately  described,  and  its  features  are  not  included  in  these 
remarks. 


PSETJDO-HTPERTKOPHIC   MtJSCTJIiAR   PaRALTSIS    (LiPOMATOUS 

MusctTLAE  Atrophy,  &c.). 

The  affection  thus  designated  usually  manifests  itself  during  the 
later  developmental  period  of  childhood,  and  clearly  depends  on  a 
morbid  developmental  tendency,  which  is  often  present  in  several 
members  of  the  same  family.  It  is  characterised  by  a  progressive 
change  in  the  size  and  diminution  in  the  power  of  many  muscles.  The 
apparent  cause  of  the  change  has  been  already  mentioned. 

Isolated  cases,  which  can  now  be  recognised  as  examples  of  this 
disease,  were  recorded  in  England  in  1830   (Sir  Charles  Bell)  and  in 


568  SPINAL   COED. 

1847  .(Partridge),  and  in  Italy  in  1838.  A  series  of  cases  was 
described  by  Meryon  in  1852,  and  Oppenheim  in  1855,  but  enlarge- 
ment of  the  muscles  was  not  conspicuous  in  these.*  Several  remarkable 
examples  had  already  come  under  the  notice  of  Duchenne,  who  was 
busy  exploring  the  field  of  muscular  paralysis  with  the  aid  of 
"  localised  electi-isation."  He  recognised  its  novel  features,  and  pub- 
lished an  account  of  it  in  1861,  under  the  name  by  which  it  has  since 
been  generally  known. 

Etiologt. — Our  knowledge  of  the  causes  of  the  disease  is  limited 
to  a  few  general  facts.  Males  furnish  the  majority  of  the  cases ;  they 
suffer  at  least  four,  and  perhaps  seven  times  as  frequently  as  females. 
In  the  latter,  moreover,  the  malady  is  slighter  in  degree,  later  in 
development,  and  less  frequently  causes  death. 

The  disease  occurs  rather  less  commonly  in  isolated  cases  than  in 
family  groups.  The  number  in  a  family  has  varied  from  two  to  eight. 
As  many  as  eight  brothers  suffered  and  died  in  the  family  described 
by  Meryon,  while  all  the  daughters  escaped.  In  a  family  known  to 
the  writer,  four  sons  have  suffered  and  none  of  the  daughters ;  in 
another  instance  two  daughters  are  affected  and  no  sons.  Thus  there 
may  be  a  tendency  in  a  family  to  the  affection  of  one  sex,  and  not  the 
other ;  but,  on  the  other  hand,  children  of  both  sexes  may  suffer  in  the 
same  family.  In  many  instances  in  which  several  members  of  o»e 
generation  are  affected,  no  antecedent  cases  can  be  traced  in  the  family ; 
the  malady,  while  congenital,  is  not  hereditary.  In  other  families 
antecedent  cases  can  be  traced,  and  these  are  invariably  on  the  mother's 
side.  The  disease  is  thus  transmitted  by  women  who  are  not  them- 
selves its  subjects.  In  a  case  in  which  four  brothers  suffered,  the 
mother's  brother  and  sister  were  likewise  affected.  Again,  a  brother 
and  sister  were  diseased,  one  daughter  of  a  second  sister,  and  three 
daughters  of  a  third  sister.  In  another  instance  a  boy  suffered,  and 
his  sister,  unaffected,  had  two  sons  diseased  and  a  daughter  free,  of 
whose  children  two  sons  were  the  subjects  of  the  malady.  Thus  the 
congenital  tendency  is  exclusively  due  to  the  maternal  element  in  the 
embryo.  This  is  also  shown  by  a  fact  many  times  observed,  that  the 
children  of  the  same  woman,  by  different  husbands,  STiffer  in  the  same 
way. 

Indirect  hereditary  tendencies,  such  as  are  indicated  by  the  occur- 
rence of  diseases  of  the  nervous  system,  can  be  traced  so  rarely  that 
it  is   doubtful  if  they  have   any  influence.      Neither  the  age  of 

♦  Nevertheless  Merlon's  cases  ('Med.-Chir.  Trans.,*  1852)  certainly  belonged  to 
this  variety.  Conclusive  proof  of  the  fact  is  afforded  by  cases  in  collaterals  which 
have  come  under  the  writer's  observation  in  near  and  distant  branches  of  the  same 
family.  Other  evidence  of  the  fact  is  described  in  a  'Clinical  Lecture  on  Pseudo- 
hypertrophic Paralysis  *  (London,  Churchill,  1879).  The  chief  literature  is  referred 
to  in  that  lecture;  the  numerous  papers  that  have  appeared  since  have  added 
chiefly  to  our  knowledge  of  the  characters  of  the  allied  forms  of  idiopathic  atrophy, 
&e.,  and  the  relations  of  these  to  the  pseudo-hypertrophic  variety,  and  are  referred 
to  on  a  later  page. 


PSEUDO-HTFERTROPHIO    MUSCULAR    PARALYSIS.  569 

parents,  nor  their  intemperance,  appears  influential,  and  their  con- 
sangtdnitj  becomes  effective  only  when  raised  in  energy  by  repetition. 
Thus,  in  a  family  known  to  me,  the  intermarriages  during  five  genera- 
tions were  very  numerous,  and  of  eight  children  in  the  present  family 
five  are  albinos,  and  two  of  these  are  the  subjects  of  pseudo-hyper- 
trophic  paralysis.  In  another  family  of  eight,  whose  parents  were 
double  first  cousins,  six  children  suffered  from  muscular  dystrophy^ 
different  forms  being  represented  in  different  members  of  the  family. 

The  disease  always  manifests  itself  during  the  period  of  development, 
sometimes  in  the  early  stage  of  growth,  at  the  close  of  infancy,  often 
only  during  mid-childhood,  rarely  not  until  growth  is  nearly  ended. 
In  a  third  of  the  cases  the  first  symptoms  are  noted  when  the  child 
first  attempts  to  walk,  which  is  usually  a  little  later  than  in  healthy 
children ;  very  rarely  indeed  the  child  has  never  walked.  In  about 
another  third  the  child  seems  well  until  it  is  four,  five,  or  six  years 
old,  and  then  impairment  of  power  attracts  attention.  In  three 
quarters  of  the  cases  the  disease  manifests  itself  before  the  tenth 
year.  Rarely  the  patient  is  conscious  of  no  symptoms  until  after 
puberty,  at  the  age  of  eighteen  or  twenty ;  but  in  such  apparently  late 
onset  there  has  been  enlargement  of  muscles  long  before  power 
became  impaired,  and  the  disease  began  much  earlier  than  it  seemed 
to  do.  One  patient,  for  instance,  in  whom  weakness  was  only  noticed 
when  she  was  twenty,  had  been  often  "  chaffed,"  when  a  yoTing  girl  at 
school,  on  account  of  her  "  tea-kettle  calves."  Neither  social  state 
nor  general  constitutional  condition  seems  to  influence  the  occurrence 
of  the  disease,  but  its  manifestation  has  sometimes  been  apparently 
accelerated  by  influences  that  disturb  the  general  health ;  it  has  been 
first  noticed,  for  instance,  during  convalescence  from  some  general 
illness. 

Symptoms. — Impairment  of  power  usually  attracts  attention  before 
any  change  is  observed  in  the  size  of  the  muscles,  or  if  these  are 
noticed  to  be  large,  it  is  with  feelings  of  parental  pride  rather  than 
with  suspicion,  in  spite  of  the  fact  that  the  children  often  walk 
clumsily,  fall  with  ease,  and  rise  with  difficulty.  The  act  of  going 
upstairs  is  especially  difficult  to  them ;  the  child  has  to  take  hold  of 
the  banisters  and  pull  himself  up. 

The  muscles  may  at  first  present  nothing  unusual,  especially  in  slight 
cases,  or  if  the  child  is  fat,  as  is  frequently  the  case.  But  at  the 
age  of  five  or  six  years  an  unnatural  enlargement  of  certain  muscles 
is  usually  conspicuous,  especially  when  there  is  a  contrast  between 
these  and  other  muscles  which  are  small.  If  enlargement  is  almost 
universal,  it  is  ubually  great  and  conspicuous.  The  enlarged  muscles 
usually  for  a  time  become  still  larger  in  comparison  with  the  others, 
but  afterwards  they  cease  to  increase  and  ultimately  become  smaller, 
first  relatively  and  then  absolutely.  This  change  occiurs  earlier  in 
some  muscles  than  in  others,  and  it  may  result  in  a  condition  of 
distinct  atrophy. 


570 


SPINAL  COED. 


Among  muscles  that  are  most  frequently  large,  those  of  the  calf 
take  the  first  place.  They  sometimes  attain  a  remarkable  size.  I  have 
measured  a  calf  14|  inches  in  circumference  in  a  boy  of  twelve.  The 
muscles  in  front  of  the  lower  leg  are  less  frequently  enlarged,  but 
sometimes  project  beyond  the  edge  of  the  tibia.  The  extensors  of  the 


Fia.  155. 


Fio.  1 Sfi. 


Fig.  157. 


Figs.  155  and  156. — Two  brothers^  aged  four  and  seven,  suffering  from 
pseudo-hypertrophic  paralysis. 

Fia.  157. — Partial  enlargement  of  rectus,  the  vasti  being  small. 

knee  are  often  big ;  occasionally  the  rectus  or  vastus  internus  is  alone 
increased  in  size  (the  rectus  in  Fig.  157),  and  the  other  parts  may  be 
normal  or  small ;  less  frequently  all  parts  are  small.  The  flexors  of  the 
knee  commonly  escape.  The  glutei  are  frequently  conspicuously  large ; 
the  flexors  of  the  hip  are,  of  course,  inaccessible  to  observation,  but 
they  are  usually  feeble,  and  no  doubt  diseased;  there  is  generally 
enlargement  of  the  lumbar  muscles,  and  disease  of  a  peculiar  and 
important  character  in  those  of  the  shoulder. 

Of  all  the  muscles  of  the  body,  next  to  those  of  the  calf,  no  one  is 
enlarged  more  frequently  or  in  greater  relative  degree  than  the  infra- 
spinatus. It  often  stands  out  so  conspicuously  that  its  edge  is  apt 
to  be  mistaken  for  that  of  the  scapula  (Figs.  158  and  159).  The 
supraspinatus  is  sometimes  also  prominent,  but  its  condition  is  usually 
concealed  by  the  trapezius,  which  is  little  involved.  The  deltoid  is 
also  frequently  large;  the  serratus  rarely.  The  pectoralis  is  never 
enlarged,  but,  on  the  other  hand,  its  lower  half  is  wasted  or  absent 


PSEUDO-HYPERTROPHIC    MUSCULAR    PARALYSIS. 


571 


in  a  large  proportion  of  the  cases,  and  with  this  the  latissimus  dorsi, 
which  has  the  same  action  in  depressing  the  raised  arm  (see  p.  37). 
The  teres  major  may  share  the  wasting  of  the  latissimus. 

The  other  muscles  of  the  arm  suffer  in  diminishing  degree  and  fre- 
quency from  above  downwards.  The  triceps  and  biceps  are  sometimes 
enlarged,  the  former  more  frequently  than  the  latter,  but  occasionally 
only  in  one  part.     Both  these  muscles  are  sometimes  wasted.     The 

forearm  muscles  suffer  in 
only  a  small  minority  of 
the  cases,  and  the  intrinsic 
muscles  of  the  hand  usually 
escape  altogether. 

This  usual  escape  of  the 
intrinsic  muscles  of  the  hand 
affords  a  very  marked  contrast 
to  spinal  muscular  atrophy, 
in  which  they  suffer  early; 


Fi(..  15^.  Pheiulo-liyinrtrophic  paralysis. 
Ab>eiice  of  latissimus  dorsi,  enlargement 
of  infiaspii:atus.     (From  a  photograph.) 


Fig.  159. — Wasting  of  latissimns 
dorsi  and  serratus ;  enlarge- 
ment of  infraspinatus,  supra- 
spinatus,  and  fleUoid  ;  atrophy 
of  biceps  and  triceps.  (By  Dr. 
H.  R.  Spencer,  irom  a  photo- 
graph.) 


hut  no  rule  is  free  from  exception,  and  although  the  escape  of  the 
hands  in  the  idiopathic  affections  is  almost  constant,  it  is  not  quite 
invariable ;  slight  (or  commencing)  impHcation  of  the  intrinsic  muscles 
has  been  met  with  in  very  rare  instances  that  were  otherwise  typical,* 

*  E.g.  Sachs,  'New  York  Neurol.  Soc.,*  Oct.  2nd,  1888;  Baumler,  '  Sudwest. 
Neurol.  Verjamml.,'  Freiburg,  1888.  I  have  once  met  with  wasting  of  the 
extensors  of  both  phalanges  of  one  thumb,  and  enlargement  of  the  abd.  indicis  has 
been  observed  (Taylor,  Clin,  Soc,  April  24th,  1891),  and  fatty  growth  in  the  thenar 
muscles  (Berger,  'Arch.  f.  Psych.,'  Bd.  xiv). 


572 


SPINAL   CORD. 


and  quite  distinct  from  tlie  "peroneal  type"  to  be  presently  men- 
tioned. 

The  muscles  of  tlie  neck  are  very  seldom  affected,  but  I  bave  noted, 
in  a  few  cases,  wasting  of  tbe  clavicular  part  of  tbe  sterno-mastoid. 

Those  of  the  face  do  not  suffer  except  in  extremely  rare  cases 
intermediate  between  this  and  the  third  type  (see  also  Westphal, 
*  Charite  Annalen,'  1887,  xii,  p.  477),  but  in  the  .patient  shown  in 
Fig.  162  there  was  considerable  enlargement  of  the  masseters.  The 
tongue  has  been  increased  in  size  in  a  few  instances.  The  other 
muscles  supplied  by  the  cranial  nerves  always  escape. 

The  diseased  muscles  are  weak,  but  the  impairment  of  power  is  to 
some  extent  irrespective  of  the  change  in  size.  The  muscles  that  are 
abnormally  small  are  generally  weaker  than  those  that  are  abnormally 
large ;  and  in  the  latter  the  weak- 
ness increases  with  the  wasting.  In 
the  legs  the  greatest  weakness  is  in 
muscles  that  are  inaccessible  to  ex- 
amination— the  flexors  of  the  hips ; 
next  in  order  of  weakness  come  the 


FlO.  160.— Modeof  obtaining  extension  of  hips 
in  pseudo-hypertrophic  paralysis.  F,  ful- 
crum of  the  lever  formed  by  the  femur.  P, 
mean  position  at  which  the  power  is  applied 
by  contraction  of  the  quadriceps  femoris. 
W,  position  of  weight  in  the  ordinary  mode 
of  rising,  w,  the  place  to  which  part  of 
the  weight  is  transferred  by  putting  hands 
on  knees. 


Pia.  161.— Mode  of  rising  from  the 
ground  in  pseudo-hypertrophic 
paralysis. 


extensors  of  the  knee  and  the  extensors  of  the  hip.  The  muscles 
below  the  knee  usually  retain  considerable  power  for  a  long  time,  and 
the  extensors  of  the  ankle  fail  before  the  flexors.  In  the  upper  limbs 
the  depressors  of  the  arm  are  usually  alone  weakened  during  the  early 
period  of  the  disease,  but  subsequently  the  shoulder  muscles  suffer. 


PSEdDO-HYPKRTROPHIC  MUSCULAR   PARALYSIS.  573 

then  the  triceps  and  the  biceps,  while  the  muscles  that  move  the  hand 
commonly  retain  good  power  to  the  last. 

The  distribution  of  weakness  in  the  legs  causes  certain  peculiai 
defects  of  movement  which  are  very  characteristic,  and  some  are  even 
all  but  pathognomonic  of  the  disease.  The  difficulty  in  going  upstairs 
is  especially  due  to  the  weakness  of  the  extensors  of  the  knee  and  hip. 
The  defect  of  the  extensors  of  the  hip  causes  the  gait  to  have  a 
peculiar  oscillating  character,  in  which  the  body  is  so  inclined  as  to 
bring  the  centre  of  gravity  over  each  foot,  on  which  the  patient  suc- 
cessively throws  his  weight,  because  the  weak  gluteus  medius  cannot 
counteract  the  inclination  towards  the  leg  that  is  off  the  ground  unless 
the  balance  is  exact.  The  greatest  defect,  however,  is  in  the  power  of 
rising  from  the  floor,  and  the  most  characteristic  peculiarity  is  the 
m.ode  in  which  this  is  achieved,  if  it  be  still  possible,  and  no  objects 
are  near  by  which  the  patient  can  aid  himself.  He  commonly  has 
not  sufficient  power  to  extend  the  knees  when  the  weight  of  the  trunk 
is  on  the  upper  extremity  of  the  femur,  which  is  then  a  lever  in  which 
the  power,  applied  between  the  fulcrum  and  the  weight,  acts  at  least 
advantage.  He  therefore  places  his  hands  on  his  knees,  as  in  Fig.  160  ; 
and  his  arms  thus  bring  much  of  the  weight  of  the  upper  part  of  the 
trunk  on  the  femur  close  to  the  fulcrum,  between  this  and  the  power, 
which  can  then  act  at  greater  advantage.  Moreover  the  mere  weight 
of  the  head  which  is  in  front  of  the  arms  tends  to  aid  the  extension. 
This,  indeed,  may  effect  the  extension  of  the  knee  without  the  aid  of 
the  extensor  muscles,  as  any  one  may  ascertain  by  observing  the 
mobility  of  the  patella  in  this  attitude.  When  the  knees  are  extended 
the  power  of  the  extensors  of  the  hip  may  be  sufficient  to  raise  the 
body  into  the  upright  position,  or  the  patient  may  aid  them  by  an 
upward  push  with  the  hand  as  he  takes  it  off.  If,  however,  these 
extensors  are  weak,  the  hands  are  often  moved  higher  and  higher  up 
the  thighs,  grasping  alternately,  and  thus  pushing  up  the  trunk.  To 
get  thus  the  requisite  support,  the  knees  must  not  be  quite  extended  ; 
and  if  their  extensors  have  no  power  the  device  cannot  be  employed, 
and  the  patient  is  altogether  unable  to  rise.  In  many  cases,  especially 
when  extension  of  the  hip  is  easy,  the  patient  achieves  the  extension 
of  the  knees  in  another  way;  he  puts  the  hands  on  the  ground, 
stretches  out  the  legs  behind  him  far  apart,  and  then,  the  chief  weight 
of  the  trunk  resting  on  the  hands,  by  keeping  the  toes  on  the  ground 
and  pushing  the  body  backwards,  he  manages  to  get  the  knees 
extended,  until  the  trunk  is  supported  by  the  hands  and  feet,  all 
placed  as  widely  apart  as  possible  (Fig.  161,  2).  Next  the  hands  are 
moved  alternately  along  the  ground  backwards,  so  as  to  bring  a  larger 
portion  of  the  weight  of  the  trunk  over  the  legs.  Then  one  hand  is 
placed  upon  the  knee  (Fig.  161,  3),  and  a  push  with  this,  and  with 
the  other  hand  on  the  ground,  is  sufficient  to  enable  the  extensors  of 
the  hip  to  bring  the  trunk  into  the  upright  position. 

The  shortening  and  contraction  of  certain  muscles  lead  to  another 


574 


SPINAL   COED. 


group  of  symptoms — distortions  due  to  permanent  alteration  in  the 
position  of  joints.  Some  of  these  are  produced,  as  are  distortions  in 
other  forms  of  muscular  weakness,  by  shortening  of  the  less  affected 
opponents  of  the  weaker  muscles.  Thus  the  knee-joints  become  fixed 
by  the  contraction  of  the  flexors,  and  the  elbow  by  the  contraction  of 
the  biceps  when  the  triceps  has  lost  all  power.  These  contractions 
only  occur  late,  and  are  usually  facilitated  by  the  habitual  flexion  of 
the  knee-  and  elbow-joints.  But  the  deformity  at  the  ankle-joint,  which 
results  from  contraction  of  the  calf  muscles,  commences  earlier,  before 
their  opponents  are  weak,  and  is  the  residt  of  shortening  of  the  muscles. 
As  a  consequence  of  it,  the  patient  cannot  get  the  heels  well  u  pon  the 
ground,  and  the  foot  cannot  be  flext  ^.  passively  beyond  a  right  angle. 
The  gradual  increase  of  the  contraction  results  in  considerable  "  talipes 
equinus ;  "  and  as  power  lessens,  the  patient  is  able  to  walk  less,  and 
the  consequent  loss  of  the  extension  involved  in  the  act  permits  a 

rapid  increase  in  the  contrac- 
tion. Tiie  feet,  as  Fig.  162 
shows,  soon  assume  a  pos- 
ture of  extreme  extension,  the 
dorsum  being  in  a  line  with 
the  front  of  the  leg,  or  forming 
with  it  a  convex  curve.  A 
subluxation  of  the  ankle-joint 
takes  place,  and  the  articular 
Burface  of  the  astragalus,  its 
anterior  extremity,  and  that 
of  the  OS  calcis,  form  three 
prominences  under  the  skin. 
When  this  reversal  of  the 
ankle  occurs,  the  tibialis  anti- 
cus  can  no  longer  act  as  a 
flexor. 

Another  deformity,  which  is 
due  chiefly  to  muscular  weak- 
ness, is  curvature  of  the  spine. 
An  antero-posterior  curve,  with 
the  concavity  backwards,  is  an 
early  symptom  of  the  disease, 
and  it  may  become  extreme, 
the  upper  part  of  the  trunk  being  carried  so  far  back  that  a  vertical 
line  from  the  scapula  falls  an  inch  or  more  behind  the  sacrum.  It 
is  due  not  to  the  weakness  of  the  trunk  muscles,  but  to  that  of  the 
extensors  of  the  hip,  in  consequence  of  which  the  pelvis  is  inclined 
forwards,  carrying  with  it  the  lower  lumbar  vertebrae ;  hence  the  upper 
part  of  the  trunk  has  to  be  held  far  back  to  keep  the  centre  of  gravity 
of  the  body  over  the  feet.  The  proof  of  this  mechanism  is  that  when 
the  patient  sits,  and  the  pelvis  is  supported  on  the  ischial  tuberosities, 


Flo.  162. — Late  stage  of  pseudo-hjperlro- 
phic  paralysis;  a  boy  fourteen  years  old, 
with  muscular  contraction  and  wasting, 
and  lateral  curvature  of  the  spine. 


PSEUDO-HYPEfiTROPHIO    MUSCULAR    PAKALTSIS. 


575 


the  lordosis  disappears.  It  is,  indeed,  replaced  by  an  opposite  curve, 
in  which  the  back  becomes  convex,  clearly  due  to  the  weakness  of  its 
extensor  muscles.  This  curve  may  become  very  great,  as  in  the  case 
shown  in  Fig.  163.  The  weakness  of  the  spinal  muscles  also  permits 
the  occurrence  of  lateral  curvature  (Fig.  102),  influenced,  in  its  direc- 
tion, by  the  habitual  posture  and  the  preponderance  of  weakness  on 
one  side  or  the  other. 


Pig.  163.— Lad  aged  fifteen ;  late  stage ;  wasting  of 
thighs;  inahility  to  sit  upright  in  consequente  of 
the  weakness  of  the  spinal  musclea. 

The  electric  irritability  of  the  muscles  is  only  altered  when  distinct 
weakness  or  wasting  has  set  in,  when  it  is  lowered  alike  to  f aradism 
and  voltaism.     There  is  never  any  trace  of  degenerative  reaction. 

The  knee-jerk  may  be  at  first  normal,  but  as  the  extensors  of  the 
knee  become  feeble,  it  is  always  lessened  and  gradually  disappears.  It 
is  never  excessive,  and  in  all  advanced  cases  it  is  lost.  Sensation  is 
unaffected,  and  so  also  are  the  sphincters  in  the  vast  majority  of  cases 
Very  rarely  there  has  been,  towards  the  end,  a  slight  difficulty  in  the 
retension  or  expulsion  of  urine,  to  be  regarded,  perhaps,  rather  as  a 
complication  than  as  an  effect  of  the  disease.  All  other  functions  of 
the  nervous  system  are  commonly  normal,  including  those  of  the  sym- 
pathetic. The  mental  development  of  the  subjects  of  this  disease  is 
generally  beyond  that  of  other  children  of  the  same  age,  doubtless  on 
account  of  the  indirect  influence  of  a  malady  which  withdraws  them 
from  active  amusements  ;  mental  defect  is  a  pure  complication. 

The  rate  of  progress  of  the  disease  and  its  duration  vary  much. 
After  some  years,  often  between  ten  and  fourteen,  the  power  of  standing 
becomes  lost  in  consequence  of  the  increasing  weakness  and  the  con- 
traction of  the  calf  muscles.  When  the  patient  ceases  to  walk  the 
muscular  disease  makes  more  rapid  progress,  deformities  become 
greater,  and  the  patient  may  become  almost  helpless,  except  in  the 
hands,  and  jet  live  on  for  several  years.      Death  is  sometimes  due  to 


676 


SPINAL   CORD. 


some  intercurrent  malady,  as  an  acute  specific  disease;  but  generally 
tlie  lessened  respiratory  power  causes  some  chest  affection  to  develop, 
or  one  tliat  should  be  trifling,  to  become  grave.  Life  is  thus  ended  by 
acute  pneumonia  or  bronchitis,  or  by  chronic  lung  disease — a  form  of 
pneumonic  phthisis  or  broncho-pneumonia,  which  develops  gradually, 
with  little  febrile  disturbance.  There  is  never  sufficient  paralysis  of 
the  respiratory  muscles  to  cause  death  directly.  The  duration  of  the 
stage  of  helplessness  depends  very  much  on  the  care  which  the  patient 
can  obtain. 

In  the  cases  in  which  muscular  power  remains  good  until  after 
puberty,  the  progress  of  the  disease  is  generally  slow.  The  patient 
may  reach  the  age  of  thirty  before  power  is  much  impaired.  It  is  possi- 
ble that,  in  some  cases,  the  disease  never  attains  a  considerable  degree. 
More  frequently,  however,  after  slight  symptoms  have  lasted  for  some 
years,  a  rapid  increase  occurs,  and  very  few  patients  reach  the  age  of 
forty.  The  course  of  the  disease  is  slower  in  girls  than  in  boys,  and 
females  furnish  a  relatively  large  proportion  of  the  late  cases. 

Varieties. — The  chief  varieties  of  the  disease  depend  on  the  age  at 
which  it  commences,  and  on  the  condition  of  the  muscles,  whether  they 
are  large  or  small.  In  rare  cases  a  single  muscle  may  be  large,  and 
tbe  rest  small,  as  in  Fig.  164,  in  which  only  the 
vasti  were  increased  in  size.  Or  everywhere  and 
f roxa  the  first  the  muscles  are  smaller  than  nor- 
mal, and  they  progressively  waste.  Such  cases 
are  not  uncommon ;  in  the  first  group  of  cases 
described  (by  Meryon),  enlargement  of  muscles 
was  inconspicuous.  Many  cases  in  which  all 
muscles  are  small  belong  properly  to  the  form 
considered  in  the  next  section.  But  it  must  be 
remembered  that  there  are  intermediate  cases 
which  form  links  between  the  two  chief  types. 
The  cases  described  by  Meryon  must  be  regarded 
as  examples  of  the  pseud o-hypertrophic  form  (see 
p.  568),  but  they  present  many  points  of  resem- 
blance to  the  "  simple  atrophy  "  described  further 
on. 

Complications. — Congenital  mental  weakness, 
due  apparently  to  defective  development  of  the 
brain,  sometimes  complicates  pseudo-hypertrophic 
paralysis.  In  rare  cases  there  have  been  indica- 
tions of  some  other  morbid  condition  of  the  central  nervous  system^ 
such  as  epilepsy.  It  is  uncertain  in  what  hght  the  slight  occasional 
affection  of  the  bladder  is  to  be  regarded,  whether  as  an  invasion  of 
the  vesical  muscles  or  as  a  central  complication.  Yigoroux  has  recorded 
a  case  in  which  the  symptoms  of  pseudo-hypertrophic  paralysis  were 
combined  with  the  peculiar  rigidity  of  Thomsen's  disease.  Of  course 
the  subjects  of  the  disease  are  liable,  like  other  children,  to  various 


Fig.  164.— Enlarge- 
ment of  the  vasti 
and  not  of  the 
rectus.  In  this  case 
all  other  muscles 
were  below  normal 
size. 


PSEUDO-HYPEKTROPHIC    MUSCULAR    PARALYSIS. 


577 


affections  of  the  nervous  system ;  I  have  seen  both  chorea  and  polio- 
myelitis as  merely  accidental  complications. 

Pathological  Anatomy. — It  is  rare  at  the  time  of  death  for  any 
muscles  to  be  actually  larger  than  natural.  Sometimes,  however,  they 
are  enlarged,  and  the  fibres  themselves  have  been  found  hypertrophied, 
just  as  they  have  been  in  excised  parts.  There  has  also  been  found 
a  great  increase  in  the  nuclei,  atrophy  of  the  fibres,  vacuolation, 
and  division  of  fibres.  Most  of  those  that  are  affected  are  below 
the  normal  size.  They  are  pale  and  yellowish  in  colour,  and  often,  to 
the  naked  eye,  resemble  perfectly  masses  of  adipose  tissue.  The 
resemblance  is  not  merely  one  of  aspect.  As  seen  under  the  micro- 
scope, it  may  be  difficult  for  the  observer  to  realise  that  he  is  not 
looking  at  a  fatty  tumour.  Nothing  may  be  at  first  visible  but  fat- 
cells,  precisely  like  those  of  adipose  tissue.  Among  the  cells,  however, 
are  tracts  of  nucleated  fibrous  tissue,  and  a  closer  examination  of  these 
shows  that  the  tracts  contain  also  muscular  fibres  (Fig.  165),  most  of 


Fl9.  166. — GastrocneTnins  muscle;  muscular  fibres,  irregularly  nar- 
rowed and  in  part  degenerated,  lie  among  tracts  of  nucleated  fibrous 
tissue,  separated  by  adipose  tissue. 


them  much  narrower  than  normal.  They  are  also  irregular  in  width ; 
a  broad  fibre,  for  instance  (as  in  the  figure),  suddenly  becoming 
narrow. 

The  fibres  for  the  most  part  preserve  their  transverse  striation,  but 
where  they  are  narrowest  this  may  have  in  part  disappeared,  either  by 
granular  degeneration,  or,  more  commonly,  by  a  simple  fading  of  the 
strisB.  In  the  narrowed  fibres  the  strise  are  sometimes  farther  apart 
than  normal.    In  other  parts  broad  fibres  may  be  seen,  normal  or 

VOL.  I.  37 


578 


SPINAL    COED. 


nearly  normal  in  aspect  (Fig.  166),  coursing  among  tlie  fat-cells,  and 
accompanied  by  a  smaller  amount  of  fibrous  tissue.  Fibres  occasion- 
ally present  fatty  degeneration,  a  longitudinal  striation  or  fissuring, 
vitreous  ("waxy")  degeneration,  or  vacuolation,  but  tliese  are  rare. 
Some  empty  sarcolemma  sbeatlis  may  be  seen  where  tlie  narrowing  of 
the  fibres  is  greatest.  In  muscles  that  still  preserve  some  red  tint 
the  amount  of  fat  is  less,  and  there  is  often  a  relatively  larger  amount 
of  interstitial  fibrous  tissue.  Yery  rarely,  in  some  part  of  a  muscle 
there  has  been  only  wasting  of  the  fibres,  without  the  interstitial 
change,  present  elsewhere  (Singer,  in  the  triceps). 


Fia.  166. — Gastroonemina  muscle;  two  nearly  normal  muscular  fibres, 
accompanied  by  fibrous  tissue,  surrouuded  by  fat-cells. 

In  some  of  these  the  interstitial  tissue  may  be  almost  entirely 
fibrous,  a  few  fat-cells  only  being  visible  here  and  there.  In  such 
muscles  it  is  common  to  find  the  fibres  more  damaged  than  in 
those  in  which  the  growth  is  partly  fatty.  It  is  chiefly  in  the  latter 
that  many  normal  fibres  are  seen.  Muscular  fibres  larger  than  normal 
are  sometimes  found  after  death.  Such  fibres  have  been  seen  in  frag- 
ments removed  during  life  by  excision,  or  by  a  "  harpoon- trocar."  It 
has  been  stated  that  the  increased  size  was  perhaps  due  to  a  vital 
contraction  under  the  mechanical  stimulus  involved  in  the  extraction. 
Sometimes,  however,  the  enlargement  has  been  unquestionable  (see 
note,  "Muscular  Hypertrophy"). 

The  motor  nerves,  when  examined,  have  been  found  normal.  The 
■condition  of  the  sensory  muscle-nerves  (which  terminate  in  the 
interstitial  tissue  in  which  the  primary  morbid  process  occurs)  has  not 
been  ascertained  in  any  instance.  The  spinal  cord  has  been  found 
perfectly  normal  in  most  cases  in  which  it  has  been  examined.  In  a 
^ew  there  have  been  slight  and  irregular  degenerative  changes,  as  in 
one  examined  by  Lockhart  Clarke  and  myself  ;*  but  the  anterior  grey 

•  *Med.-Chir.  Trans.,'  vol.  Ivii,  p.  247.  In  this  case  the  cervical  and  dorsal 
regions  were  normal,  with  the  exception  of  here  and  there  slight  accumulationg,  at 


PSEUDO-HTPEETEOPHIC    MUSCULAE   PAEALTSIS,  579 

matter  was  unaffected,  and  the  changes  were  probably  merely  asso- 
ciated, and  not  the  canse  of  the  symptoms.  Haemorrhages  have  been 
occasionally  found.  Probably  they  have  occurred  late  in  life  in  the 
degenerated  tissue.  The  neuroglial  cells  have  been  found  increased  in 
number,  and  the  fibres  of  the  white  substance  have  been  found  irregu- 
larly changed,  in  a  few  cases,  without  any  constancy  in  the  seat  of  the 
alteration. 

PATHOLoaT. — The  common  integrity  of  the  anterior  grey  matter  of 
the  cord,  and  especially  of  the  motor  nerve-cells,  seems  conclusive 
evidence  that  the  disease  of  the  muscles  is  not  due  to  a  primary  lesion 
of  the  spinal  cord.  The  slight  irregular  changes  occasionally  found 
are  probably  consecutive  to  the  long  inaction  of  the  cord  and  deformity 
of  the  spine.  Pseudo-hypertrophic  paralysis  is  not,  therefore,  as  was 
at  first  thought,  merely  a  form  of  spinal  musctdar  atrophy  with  a 
special  muscular  change.  The  significance  of  the  pathological  anatomy 
is  that  the  malady  is  a  primary  disease  of  the  muscles,  consisting  in 
an  altered  condition  of  the  muscular  fibres,  leading,  it  may  be,  to 
enlargement  and  subsequently  to  wasting  and  associated  overgrowth 
of  the  connective  tissue,  in  which  fat  may  or  may  not  be  deposited. 
The  indication  of  the  conditions  under  which  the  disease  occurs  is 
that  it  is  congenital,  the  result  of  a  perverted  tendency  of  growth, 
inherent  in  the  embryo,  and  derived  from  the  germ  from  which  the 
embryo  proceeds.  In  this  connection  it  is  instructive  to  note  that 
there  is  one  form  of  congenital  tumour,  the  structure  of  which  is 
almost  exactly  the  same  as  that  of  the  muscles  in  pseudo-hypertrophic 
paralysis.  Fig.  167  might  be  a  fragment  of  a  muscle  in  this  disease, 
but  it  is  a  section  of  a  myolipoma,  the  congenital  character  of  which 
is  emphasised  by  the  fact  that  it  was  attached  to  the  conus  meduUaris 
of  the  spiaal  cord  of  a  patient  whose  muscles  were  healthy.  (The 
tumour  is  shown  also  in  Fig.  174.)  It  must  have  been  due  to  the 
misplacement  of  some  of  the  embryonal  elements  from  which  muscular 
tissue  is  developed,  and  it  shows  that,  from  such  elements,  the  struc- 
tural condition  found  in  pseudo-hypertrophic  paralysis  may  arise. 
The  points  of  chief  importance  in  the  general  pathology  of  the  disease 
have  been  mentioned  in  the  introductory  remarks.  The  difficult  ques- 
tion of  the  precise  mechanism  by  which  the  muscular  fibres  suffer 
must  be  regarded  as  an  open  one.  We  may  assume  a  defective  vitality 
in  them,  but  the  conspicuous  lesion  is  the  growth  of  connective  tissue, 
and  by  this  the  fibres  are  doubtless  damaged,  whether  fatty  tissue  is 
formed  or  not.    Indeed,  the  fibres  seem  to  suffer  more  when  there  is 

the  bottom  of  the  fissures,  of  products  of  degeneration,  probably  derived  from  the 
perivascular  erosion  common  at  all  ages.  At  the  last  dorsal  segment,  however,  there 
was  an  area  of  granular  disintegration  in  the  intermediate  grey  substance  on  each 
side,  in  front  of  the  posterior  vesicular  tract.  This  pwrt  was  unduly  translucent  for 
half  a  centimetre  in  vertical  extent,  and  in  the  middle  of  this  area  the  disintegration 
bad  produced  an  actual  cavity,  across  which  the  fibres  for  the  cerebellar  tract  ran 
unchanged. 


580 


SPINAL   CORD. 


only  fibrous  tissue  than  when  there  is  the  fatty  deposit,  although  it  is 
chiefly  the  fat  that  causes  the  enlargement  of  the  muscles.  There 
seems  to  be  a  tendency  to  the  formation  of  fat  in  the  early  stage  of  tbe 
disease,  and  to  its  removal  in  the  later  stage,  since  in  the  former  the 


■^2277 


Fia.  167. — Section  of  a  myolipoma  which  was  attached  to  the  spinal 
cord  ot  a  man  suffering  from  lociomotor  ataxy,  for  comparison  with 
Figs.  165  and  166. 

muscles  may  often  be  observed  to  increase  in  size,  and  in  the  latter 
they  become  smaller,  partly  from  some  removal  of  fat  and  partly  from 
the  increasing  atrophy  of  the  muscular  fibres.  It  is  then  that  the 
greatest  impairment  of  power  occurs.  In  the  cases  in  which  the 
muscles  are  small  from  the  first,  the  tendency  to  the  deposit  of  fat 
seems  slight,  and  the  condition  is  an  almost  pure  muscular  sclerosis. 
The  late  shortening  which  occurs  seems  to  be  due  to  the  contraction 
of  the  interstitial  fibrous  tissue,  and  it  often  coincides  with  the 
shrinkage  from  the  removal  of  some  of  the  fat,  and  the  atrophy  of  the 
muscular  fibres. 

.  If  it  be  true  that  the  latissimus  dorsi  and  lower  half  of  the 
pectoralis  are  sometimes  congenitally  absent  in  pseudo-hypertrophic 
paralysis,  the  fact  is  quite  consistent  with  the  explanation  of  the 
nature  of  the  disease  given  above.  These  two  muscles  stand  perhaps 
lowest,  in  functional  importance,  among  the  muscles  of  the  body, 
being  used  chiefly  for  the  rare  depression  of  the  arm  against  a 
resistance  (see  p.  37).  It  is  readily  intelligible  that  a  defect  in  the 
embryonal  tissue  of  the  muscular  system  should  be  quantitative  as 
well  as  qualitative. 

The  loss  of  the  knee-jerk  is  sufficiently  explained  by  the  lesion  of 
the  muscles.  According  to  the  theory  that  the  irritability  on  which 
the  jerk  depends  is  due  to  a  muscle-reflex  action,  the  loss  is  readily 
intelligible,  since  the  afferent  influence  is  due  to  the  stimulation,  by 
tension,  of  the  sensory  muscle-nerves,  and  these  end  (or  begin)  in  the 
interstitial  tissue,  which  is  the  seat  of  the  primary  morbid  process  in 


pseudo-hyperteophk;  musculae  paralysis.         581 

this  disease.  But  the  wasting  of  the  muscular  fibres  must  also  be 
capable  of  abolishing  the  knee-jerk  when  the  atrophy  reaches  a 
considerable  degree,  and  it  is  also  possible  that  the  motor  nerve- 
endings  share  this  structural  damage.  On  any  theory  of  the  nature  of 
the  knee-jerk,  this  will  explain  its  loss.  It  is  only  when  the  muscular 
changes  have  attained  a  considerable  degree  that  the  loss  occurs. 
The  fact  therefore  does  not,  in  itself,  suggest  any  lesion  of  the  spinal 
cord.  If  the  malady  is  thus  one  of  a  morbid  developmental  tendency, 
it  is  not  surprising  that  a  tendency  to  analogous  developmental 
diseases  should  be  sometimes  present  in  the  nervous  system,  and  that 
thus  imbecility  and  epilepsy  on  the  one  hand,  or  albinism  on  the  other, 
should  be  occasionally  associated  with  the  disease. 

Diagnosis. — The  diagnosis  of  the  disease  is  usually  easy  if  its 
characters  are  known.  The  peculiarity  of  gait  and  the  mode  of  rising 
from  the  floor,  the  age  of  the  patient,  and  the  progressive  character  of 
the  impairment,  are  in  themselves  sufficient  to  suggest  the  affection, 
and  examination  reveals  enlargement  and  often  contraction  of  the  caK 
muscles,  and  a  change  in  the  size  of  others,  which  confirm  the  diagnosis. 
The  mode  of  rising  is  not  absolutely  pathognomonic,  and  it  has  misled 
even  good  observers ;  it  is  occasionally  acquired  in  other  diseases  in 
which  there  is  a  gradual  weakening  of  the  extensors  of  the  hip  and 
knee.  It  is  met  with  equally  in  the  simple  idiopathic  atrophy,  but 
is  so  rare  from  any  other  cause  as  to  be  of  very  great  diagnostic 
suggestiveness. 

Of  the  condition  of  the  muscles,  that  which  is  most  characteristic 
is  the  combination  of  enlargement  of  the  infraspinatus  with  a 
wasting  of  the  latissimus  and  lower  part  of  the  pectoralis.  I  pointed 
out  some  years  ago  *  that  this  condition,  which  is  seldom  absent,  is  of 
very  high  diagnostic  importance,  and  subsequent  observations  have 
fully  confirmed  the  opinion,  Next  in  importance  is  the  enlargement 
of  the  calf  muscles,  especially  in  combination  with  contracture  that 
cannot  be  overcome.  In  general,  the  enlargement  and  diminution  of 
neighbouriag  muscles  is  very  significant,  but  it  must  be  remembered 
that  they  may  be  gravely  diseased,  and  yet  of  normal  size,. or  from 
the  first  smaller  than  normal.  In  such  cases  they  are  often  hard, 
and  the  distribution  of  the  affection  is  the  same  as  that  of  the  double 
change  in  typical  cases.  Such  cases  illustrate  the  relation  of  this 
form  to  the  simple  atrophy  described  in  the  next  section. 

The  disease  with  which  confusion  is  most  common  is  the  so-caUed 
"congenital  spastic  paraplegia"  (p.  495).  Both  diseases  affect 
children ;  in  both  there  are  weakness  of  the  legs  and  contraction  of  the 
calf  muscles,  and  in  both  the  muscles  are  frequently  large.  The  chief 
distinctions  have  been  already  mentioned.  The  most  important  are 
the  preservation  and  excess  of  the  knee-jerk  in  spastic  paraplegia,  the 
tendency  to  spasm  of  the  legs — the  facts  that  the  contracture  is  active 
and  can  be  overcome,  that  the  patient  does  not  rise  from  the  ground 
*  *  Pseudo-hypertrophic  Muscular  Paralysis,*  London,  1879. 


582  SPINAL    COKD. 

in  the  way  peculiar  to  pseudo-typertropMc  paralysis, — and  the  oppo- 
site tendency  of  the  two  diseases.  Congenital  dislocation  of  both 
hips  sometimes  presents  a  superficial  resemblance.  Spinal  muscular 
atrophy  is  only  likely  to  be  confused  with  the  simple  atrophy. 

Between  the  two  forms  of  idiopathic  atrophy  the  distinction  is 
scarcely  one  of  diagnosis  proper  ;  it  is  rather  a  question  of  the  category 
in  which  a  case  should  be  placed.  The  most  important  distinction  is 
the  freedom  of  the  caK  muscles  from  enlargement  in  the  atrophic  form. 
The  face  is  not  affected  in  pseudo-hypertrophy  as  it  often  is  in 
idiopathic  atrophy,  except  only  in  some  intermediate  instances.  If 
more  than  one  member  of  a  family  is  affected,  some  of  the  sufferers 
will  usually  present  characteristic  symptoms  of  the  special  form,  for  it 
is  remarkable  with  what  constancy  the  two  types  generally  remain 
distinct  (see  p.  567). 

Pkognosis. — In  the  case  of  any  child  with  pseudo-hypertrophio 
paralysis,  the  prognosis  is  most  grave.  It  is  almost  certain  that  each 
year  will  bring  increasing  disability,  and  that  the  patient  will  not 
reach  adult  life.  It  is  only  when  the  disease  develops  late,  and  the 
symptoms  do  not  become  considerable  until  after  twenty  years  of  age, 
that  there  is  a  possibility  that  the  disease  may  not  attain  its  ultimate 
degree,  but  even  in  such  cases  this  hope  is  seldom  realised.  In  any 
case,  and  at  any  age,  it  is  unhkely  that  the  patient  will  live  more  than 
seven  years  after  the  power  of  standing  is  lost.  But  even  in  this  con- 
dition arrest  seems  to  take  place  in  the  progress  of  the  disease.  One 
patient  is  known  to  the  writer  who  presents  a  typical  picture  of  the 
pseudo-hypertrophic  type,  who  is  now  over  forty,  and  has  never  been 
able  to  run.  He  has  remained  stationary  certainly  during  the  last 
eight  years,  probably  longer. 

Treatment. — As  a  congenital  developmental  malady,  pseudo-hyper- 
trophic paralysis  is  one  of  those  diseases  in  which  medicine  is  neces- 
sarily powerless  to  cope  with  the  essential  elements  of  the  process. 
As  may  be  therefore  expected,  no  drug  has  been  found  to  exert  an 
influence  on  the  course  of  the  affection,  although  such  nervine  tonics 
as  phosphorus  and  arsenic  have  been  thought  sometimes  to  retard  for 
a  time  the  progress  of  the  weakness.  The  stimulation  of  the  muscles  by 
electricity  has  been  employed  and  advocated,  but,  howeTOr  sedulously 
employed,  no  distinct  result  follows  the  use  of  either  faradism  or 
voltaism.  It  must  be  remembered,  moreover,  that  electricity  is  a  very 
feeble  agent  in  stimulating  the  growth  of  muscular  fibres,  compared 
with  the  physiological  stimulus  of  voluntary  effort.  Muscular  exercise 
may  reasonably  be  looked  to  in  order  to  make  up,  in  some  degree, 
what  is  lacking  in  this  disease,  and  does  seem  to  have  some  influence 
in  retarding  the  failure  of  power.  It  may  perhaps  induce  further 
growth,  or  greater  power  in  the  muscular  fibres  that  have  not  yet 
suffered,  or  actually  supplement  the  defective  trophic  energy.  When 
muscular  exercise  is  stopped,  there  is  certainly  a  quicker  failure  of 
strength.     Hence  it  is  desirable  that  the  patient  should  carry  out 


SIMPLE   IDIOPATHIO  MUSCULAR    ATROPHY.  588 

carefully  planned  gymnastic  exercises,  so  arranged  as  to  call  into 
action  tne  muscles  that  most  need  help.  These,  thoroughly  per- 
severed in,  have  seemed,  more  than  any  other  means,  to  retard  the 
disease.  Although  they  have  not  in  any  case  arrested  it,  the  trouble 
that  is  necessary  to  arrange  the  method  is  certainly  well  spent. 
Eubbing  and  massage  improve  the  circulation,  and  help,  especially 
when  combined  with  passive  movements,  to  lessen  the  tendency  to 
muscular  contraction  and  consequent  deformities. 

The  influence  of  muscular  exercise  renders  it  very  important  to 
maintain  locomotion  as  long  as  possible.  The  ability  to  stand  and 
walk  is  generally  lost,  through  the  contraction  of  the  calf  muscles, 
some  time  before  the  muscular  weakness  would  take  the  patient  off  his 
feet.  In  such  cases  tenotomy  may  restore  the  power  of  walking  for 
some  years,  and  when  the  contracture  returns,  its  removal  has,  a 
second  time,  enabled  walking  to  be  resumed.  The  operation  is  thus 
distinctly  beneficial,  and  should  be  performed  as  soon  as  the  actual 
need  for  it  arises,  and  division  of  the  tendon  is  far  better  than  any 
imperfect  substitute. 

During  the  later  stages  of  the  disease  great  care  is  required  to 
preserve  the  patient  from  catarrh,  which  helps  to  excite  the  pulmonary 
mischief  that  so  often  ends  life.  Similar  care  is  also  needed  during 
any  intercurrent  malady  which  the  patient  may  contract. 


Simple  Idiopathic  Muscular  Atrophy.* 

The  cases  in  which  there  is  no  muscular  enlargement,  in  which 
wasting  is  manifest  from  the  first  in  the  size  of  the  muscles,  are  much 
more  rare  than  the  pseudo-hypertrophic  disease.  They  belong  to 
several  types,  more  or  less  distinct,  of  which  the  most  common  is  that 
to  which  Erb  has  given  the  name  of  "  juvenile,"  and  of  which  most 
instances  are  probably  the  pseudo-hypertrophic  disease  without  mus- 
cular enlargement;  others  are  more  special,  since  the  lower  limb  muscles 
have  suffered  but  little.  A  more  striking  variety  is  that  which  is 
characterised  by  the  affection  of  the  muscles  of  the  face  in  addition  to 
those  of  the  shoulder  girdles — the  "  facio-scapulo-humeral  type  "  of 

*  The  most  important  writings  on  the  subject  (besides  those  specially  quoted)  are 
those  of  Duchenne  in  'Electrisation  localise'  (p.  60of  Poore's  translation,  published 
by  the  New  Sydenham  Society);  Barsickow,  'Inaug.  Dissert.,' Halle,  1872  j  Leyden, 

*  Klin.  d.  Ruckenm.  Krank.,'  Bd.  ii,  p.  525 ;  Mobius,  "  Hered.  Nervenk.,"  Volkmann'a 

•  Klin.  Vortrage,' No.  171 ;  Landonzy  and  Dejerine, '  Revue  de  Med.,'  1885,  pp.  81 
and  251 ;  Marie  and  Guinon  (a  series  of  cases  observed  at  the  Salpetriere),  lb.,  1885 ; 
Sachs,  'New  York  Med.  Journ.,'  Dec.  15tb,  1888;  Hitzig,  'Berlin,  klin.  Wochen- 
8chr.,*  1888 ;  Singer,  *  Zeitsch.  f.  Heilk..'  Bd.  viii.  Especially  valuable  are  the  papers 
by  Erb,  'Deut.  Archiv  f.  klin.  Med.,'  Bd.  xxxiv,  1884,  and  'Neurol.  Centralbl.,' 
July  Ist,  1886,  with  numerous  later  writings.  Bibliographical  references,  &e.,  will 
be  found  also  in  Tooth's  *  Thesis  on  the  Peroneal  Type,'  and  in  my  Lecture  on 
Pgeudo-hypertrophic  Paralysis  (1879),  and  in  Sachs'  article  (loc.  cit.). 


684 


SPINAL    COl?D. 


Landotizy  and  Dejerine.  In  a  tliird  variety  the  affection  begins  in 
the  legs,  and  is  peculiarly  slow  in  course.  Some  other  types  may 
ultimately  be  differentiated,  but  it  is  important  to  re(;ognise  the  fact 
that  even  those  that  we  can  now  distinguish,  as  the  most  pronounced 
in  their  features,  do  not  keep  entirely  distinct.     They  are  connected, 

. . w        " " :    " 


/ 


Fia.  168. — Simple  idiopathic  muscular  atiophy.  From  photogiaphs 

kindly  lent  by   Dr.  J.  H.  Crocker.     Particulars  of  these  cases  and 

many  similar  are  given  in  an  excellent  tiiesis  on  this  disease  by 
Dr.  Crocker. 


by  intermediate  forms,  not  only  with  each  other,  but  even  with  the 
pseudo-hypertrophic  disease,  as  already  mentioned.*  But  these  are 
■unusual ;  as  a  rule  the  two  latter  types  remain  distinct  in  the  families 
in  which  they  occur,  and  this  is  the  justification  for  their  separate 
description.  In  those  families  in  which  simple  muscular  atrophy 
occurs,  however  numerous  the  cases,  however  different  the  distribu- 
tion of  the  disease,  and  however  various  the  ages  at  which  it  begins, 
cases  rarely  present  the  distinctive  characters  of  pseudo-hypertrophic 
paralysis. 

There  is  less  constant  separation  between  the  simple  "  juvenile " 
and  the  facial  forms.  Although  they  often  keep  distinct,  the  affec- 
tion of  the  face  has  been  absent  in  a  few  cases  in  a  family  in  which  it 

•  JB.  g.  case  described  and  collected  by  Marie  and  Guinon  (loc.  cit.),  connecting 
the  pseudo-hypertrophic  form  with  both  the  juvenile  and  the  facial. 


SIMPLE    IDIOPATUIO    MUSODLAR   ATROPHY.  585 

was  the  first  part  to  be  affected  in  most  sufferers.  Thus  a  man  with 
simple  atrophy,  beginning  at  fourteen  in  the  shoulder  and  thigh 
muscles,  whose  face  was  unaffected,  had  a  daughter  who  began  to 
suffer  at  eleven,  and  in  whom  the  face,  scapular,  and  arm  muscles 
were  involved.*  The  peculiar  "  peroneal  type  "  is  not  included  in  this 
outline,  and  the  statements  here  made  do  not  ajDply  to  it. 

Causes. — We  are  able  to  trace  no  cause  beyond  the  congenital 
tendency,  already  considered,  shown  by  the  occurrence  of  many  cases 
in  the  same  family,  and  in  more  than  one  generation.  In  one  remark- 
able series  recorded  by  Barsickow,  twenty-four  cases  were  distributed 
through  five  generations,  and  the  disease  was  also  traced  through  five 
generations  in  a  group  described  by  Landouzy  and  Dejerine  It  is 
very  rare  for  the  disease  to  be  confined  to  one  generation,  far  more 
rare  than  for  pseudo-hypertrophic  paralysis  to  be  so  confined.  But, 
as  in  the  case  of  most  congenital  hereditary  diseases,  cases  that  are 
apparently  isolated  are  occasionally  met  with.  Such  isolated  cases  are 
rare — more  so,  probably,  than  in  the  pseudo-hypertrophic  form;  al- 
though wider  observation  may  show  that  they  are  more  frequent  than 
we  now  suspect.  In  the  families  of  the  patients  shown  in  Figs. 
169-172  no  example  of  analogous  disease  could  be  heard  of. 

Both  sexes  suffer ;  in  a  few  families,  females  chiefly ;  in  others, 
males  ;  in  most,  both  have  been  affected.  The  age  at  which  the  disease 
first  manifests  itseK  is  extremely  variable.  It  may  begin  as  early  as 
two  t  or  three,;}:  and  as  late  as  sixty  years.  But  the  onset  is  seldom 
during  childhood;  in  the  majority  of  cases  the  disease  shows  itself 
between  fifteen  and  thirty-five ;  that  is,  during  the  later  period  of 
growth  and  the  early  period  of  adult  life.  Even  in  the  same  family, 
the  variations  maybe  extreme;  in  that  described  by  Barsickow  tbe 
date  of  the  onset  of  seventeen  cases  was  known,  and  was  as  follows  : — 
in  one  at  12 ;  in  four  between  15  and  20 ;  in  seven  between  20  and  30 ; 
in  three  between  30  and  40  ;  in  two  after  40.  A  woman  aged  fifty-two 
began  to  suffer  at  30,  but  her  son  at  3  years  of  age.§  Sex  has  no 
influence  on  the  date  of  onset,  nor,  as  a  rule,  can  any  relation  be  traced 
between  the  date  and  the  place  at  which  the  wasting  begins.  When  the 
wasting  begins  in  the  face,  the  disease  more  frequently  commences  in 
childhood  than  when  the  first  symptoms  are  in  the  limbs,  but  in  some 
instances  the  atrophy  has  commenced  in  the  face  late  in  life,  and  this 
in  the  same  family  in  which  other  sufferers  have  been  young.  Thus 
in  the  sexual  I'roclivity,  and  in  the  date  of  onset,  there  is  a  marked 
difference  between  this  form  and  pseudo-hypertrophy,  the  latter 
showing  a  stronger  tendency  to  affect  males,  and  to  manifest  itself  in 
childhood.     The  "  juvenile  "  form,  as  a  rule,  presents  less  difference. 

*  Troisier  and  Guinon,  'Rev.  de  Med.,'  1889,  p.  48.  See  also  Singer,  *Zeit.  f 
Heilk.,'  viii,  p.  229. 

t  Kreske,  *  Munch,  med.  Wochenschr.,'  1886. 
J  Landouzy  and  Dejerine,  loc.  cit. 
§  Ibid. 


586  SPINAL    CORD. 

As  a  rule,  no  direct  exciting'  cause  can  be  traced.  In  a  few  instances 
tlie  onset  has  succeeded  some  other  morbid  process,  such,  as  chlorosis, 
acute  disease,  or  rheumatic  affections  due  to  exposure  to  cold ;  and  the 
depression  of  general  health  resulting  from  these  may  have  determined 
the  time  of  onset,  but  is  not  likely  to  have  done  more.  In  other  in- 
dividuals of  the  same  families  the  disease  has  developed  without  the 
aid  of  any  exciting  influence. 

Symptoms. — The  onset  is  always  gradual.  Weakness  and  wasting 
come  on  together,  and  are  noticed  simultaneously,  unless  the  com- 
mencing atrophy  is  concealed  by  subcutaneous  fat.  The  atrophy 
generally  begins  in  the  upper  arm  and  shoulder  muscles,  having,  in 
the  most  common  "  juvenile "  form,  a  similar  distribution  in  these 
parts  to  that  of  pseudo-hypertrophic  paralysis.  In  the  facial  form, 
however,  this  part  usually  suffers  first,  as  in  the  lad  figured  on  p.  589. 
In  rare  cases  the  wasting  not  only  begins  in  the  legs,  but  remains 
limited  or  almost  limited  to  them.  In  the  part  first  affected  the  dis- 
ease slowly  increases,  and  thence  it  usually  spreads.  The  onset  may 
be  symmetrical  on  the  two  sides,  or  one  side  may  suffer  some  time 
before  the  other. 

Of  the  arm  muscles,  the  weakness  and  wasting  are  noticed  first  in 
the  biceps  and  triceps,  and  with  these  the  supinator  longus  often 
suffers.  But  examination  generally  shows  that  the  lower  part  of  the 
pectoraHs  and  latissimus  dorsi  are  greatly  wasted,  a  loss  of  which  the 
patient  may  be  little  aware,  on  account  of  the  relative  unimportance  of 
these  muscles.  Sometimes  the  upper  part  of  the  pectoralis,  and  even 
the  pectoralis  minor,  are  also  affected.  The  tendency  to  atrophy  of 
the  lower  part  of  the  pectoralis  and  latissimus  is  a  character  which,  as 
already  stated,  is  common  to  this  disease  and  pseudo-hypertrophic 
paralysis,  but  not  invariable  in  either.  The  deltoids  are  rarely  in- 
volved ;  in  many  cases  they  are  normal ;  sometimes  they  have  been 
thought  to  be  unduly  large  ;  in  a  few  instances  they  have  been  wasted. 
The  serratus  magnus  is  often  affected  (Fig.  172),  but  may  escape,  even 
in  a  severe  case.  The  supraspinatus  and  infraspinatus  may  also 
suffer,  but  are  often  normal,  or  even  enlarged;  the  trapezius  and 
rhomboids  have  been  affected  in  many  cases ;  sometimes  much  atro- 
phied, especially  in  the  "  juvenile"  form. 

The  forearm  muscles  generally  escape,  with  the  exception  of  the 
supinator  longus.  Occasionally  there  has  been  some  weakness  of  the 
long  extensors  or  flexors  of  the  fingers,  with  or  without  slight  visible 
wasting.  In  the  case  mentioned  on  the  next  page  the  extensors  of 
the  phalanges  of  the  thumb  were  involved  on  the  left,  and  the  radial 
extensor  of  the  wrist  on  the  right  side.  Earely  the  forearm  muscles 
have  been  much  atrophied.  In  several  instances  there  has  been  some 
atrophy  of  the  small  muscles  of  the  hands,  the  thenar  and  interosseal 
muscles,  or  the  interossei  only,  as  in  the  case  of  Landouzy  and 
Dejerine ;  but  in  the  majority  the  integrity  of  these  muscles  is  a  marke-d 
feature  of  the  disease  and  a  contrast  to  the  spinal  form. 


SIMPLE    IDIOPATHIC    MUSCULAB   ATEOPHT.  587 

The  affection  of  the  face  is  peculiar.  There  is  commonly  a  failure 
of  the  zygomatic  muscles,  and,  in  consequence,  a  loss  of  the  naso-labial 
furrow,  and  a  curious  alteration  in  the  smile ;  instead  of  the  angles  of 
the  mouth  being  drawn  outwards  and  upwards,  they  are  moved 
upwards  by  the  elevators  of  the  upper  lip  and  angle  of  the  mouth. 
The  orbicularis  oris  is  also  affected,  and,  in  consequence,  the  lips 
arehabitually  separated,  the  lower  lip  projects,  the  patient  cannot 
"pout"  or  whistle,  and  the  articulation  of  labials  is  imperfect. 
The  face  has  a  dull  expression,  and  the  aspect '  is  very  pecuUar ; 
it  has  been  termed,  by  Landouzy  and  Dejerine,  the  "  myopathic 
face."  In  one  case,  observed  by  them,  the  face  was  unequally 
involved  on  the  two  sides.  Earely  (as  in  Fig.  171)  the  frontales 
have  been  involved,  and  the  orbicularis  palpebrarum  has  been 
weak,  and  the  eyes  cannot  be  completely  closed.  Weakness  of  the 
eyeUds  was  supposed  to  be  the  cause  of  distinct  exophthalmos  present 
in  one  woman.*  In  the  case  described  by  Kreske,  inability  to  close  the 
eyes  was  noted  at  three,  and  at  ten  the  paralysis  of  the  face  was 
absolute.  Wasting  of  the  muscles  may  be  indistinct,  because  the 
contour  of  the  face  is  only  to  a  slight  degree  influenced  by  the  substance 
of  the  muscles  The  projecting  lower  lip  may,  indeed,  appear  to  be 
thicker  than  normal.  In  many  cases  the  buccinators  have  been  affected, 
in  some  instances  they  have  been  normal,  and  then  have  drawn  out  the 
angles  of  the  mouth  in  smiling.  The  tongue  has  been  always  un- 
affected, and  so  also  have  the  pharynx,  larynx,  muscles  of  mastication, 
and  the  eyeball  muscles. f 

The  muscles  of  the  spine  have  sometimes  been  normal,  sometimes 
they  have  been  considerably  atrophied.^     The  intercostals  are  often 

•  Landouzy  and  Dejerine. 

t  In  a  singular  case  under  my  care  some  years  ago,  an  affection  of  the  facial 
■muscles,  similar  to  that  of  idiopathic  muscular  atrophy,  was  associated  with  paralysis 
of  ocular  muscles.  The  patient  was  a  girl  twenty-seven  years  of  nge ;  there  were  no 
indications  of  syphilis,  nnr  could  any  history  of  muscular  atrophy  in  the  family  be 
ascertained.  The  ocular  palsy  commenced  gradually  at  twenty-four,  and  increased 
until  the  movemeitts  of  both  eyes  upwards,  of  the  left  eye  inwards,  and  tlie  right  out- 
wards, were  lost,  and  all  other  movements  were  weakmed.  Thu  eyelids  drooped 
sligh  I  ly  ;  the  intei  ii:il  ocular  muscles  were  normal.  The  affection  of  the  face  followed 
tliat  of  the  eyes;  the  zygomatic  muscles  were  powerless,  so  that  the  smile  consisted 
only  in  elevation  of  the  upper  lip;  the  orbicularis  was  weak.  The  palate,  pharynx, 
and  iHiynx  were  normal.  The  arms  became  feeble,  and  the  flexors  of  the  hips 
almost  powerless.  There  was  no  visible  change  in  the  nutrition  or  electrical  irrita- 
bility of  the  muscles;  the  knee-jerk  was  normal.  If  the  case  was  central,  as  it 
appt^art'd  to  be,  the  peculiar  affection  of  the  lips  and  zygomatics  is  not  confined  to 
idi(ipnthic  muscular  atrophy.  If  the  disease  was  muscular,  the  eyeball  muscles  do 
not  invariably  escape.  With  this  case  may  be  noted  one  recorded  by  Oppenheim 
('Charite  Aniialen,'  xiii)  as  the  "juvenile"  form,  with  derangement  of  the  lateral 
movement  of  the  eyi's,  nystagmus,  and  some  laryngeal  palsy.  The  shoulder  and 
thigh  muscles  .suffered  chiefly,  but  below  the  knee,  the  peronei — a  noteworthy 
aberration  from  the  type. 

J  Atrophy  began  in  the  back  muscles  at  forty-four  in  a  case  recorded  by  Musio 


588  SPINAL    CORD. 

affected  in  the  later  stages,  but  rarely  in  extreme  degree.  .The 
diaphragm  also  sometimes  suffers.  The  abdominal  muscles  have 
bjen  involved  in  only  a  few  instances.  In  the  legs  the  muscles 
most  commonly  affected  are  the  flexors  of  the  hip,  the  extensors 
of  the  knee,  and  less  frequently  the  glutei.  The  muscles  below 
the  knee  have  escaped  in  many  cases ;  when  they  have  suffered,  the 
atrophy  has  often  been  general,  ^^'hen  the  peroneal  and  anterior 
tibial  muscle-  have  been  specially  affected,  as  in  the  case  shown  in 
Fig.  172,  the  case  has  generally  b^en  of  the  peculiar  "j^eroneal" 
type  to  be  presently  mentioned,  although  it  is  possible  that  some 
instances  of  the  kind  are  on  the  borders  of  the  group  now  under  con- 
sideration. 

The  electric  irritability  of  the  affected  muscles  is  usually  lessened 
in  proportion  to  the  wasting,  and  equally  to  both  currents.  Indeed, 
the  diminution  seems  sometimes  out  of  proportion  to  the  wasting,  and 
great  when  the  atrophy  is  slight.  There  is  no  trace  of  degenerative 
reaction,  and  there  is  not  even  the  longer  persistence  of  voltaic  than 
of  f aradic  irritability  which  occurs  in  the  more  chronic  cases  of  spinal 
atrophy.  Fibrillation  is  almost  always  absent,  but  not  quite  in- 
variably ;  hence  its  absence  cannot  be  made  distinctive,  stiU  less  its 
presence.  Myotatic  irritability  is  lessened  or  lost ;  it  is  never  increased. 
Some  shortening  of  muscles  has  been  occasionally  noted,  frequently  in 
the  calf  muscles,  rarely  in  the  biceps. 

All  other  functions  of  the  nervous  system  are  unaffected.  Sensi- 
bility is  normal.  In  a  few  cases  transient  rheumatoid  pains  have 
accompanied  a  rapid  development  of  the  disease  in  the  arms,  but  in 
the  vast  majority  of  cases  the  disease  is  painless.  The  sphincters  are 
unaffected,  and  there  is  no  tendency  to  trophic  or  vaso-motor  dis- 
turbance. 

As  the  muscular  atrophy  progresses,  the  form  of  the  affected  parts 
becomes  changed  just  as  in  spinal  atrophy.  Deformities  may  also 
occur,  chiefly  from  the  shortening  of  less  affected  muscles,  but  these 
rarely  reach  the  considerable  degree  common  in  other  forms  of 
muscular  wasting.  Lordosis  occurs  in  the  upright  posture,  and  is 
probably  due  to  the  same  mechanism  as  in  pseudo-hypertrophic 
paralysis,  ceasing,  as  in  that  disease,  when  the  patient  sits  (compare 
Figs.  169  and  172).  When  the  muscles  of  the  lower  leg  are  involved 
talipes  may  develop. 

The  course  and  duration  of  the  disease  are  exceedingly  variable. 
The  atrophy  may  remain  limited  to  the  part  in  which  it  begins.  The 
face  alone  ha  «  been  affected  in  some  members  of  a  family,  although  in 
other  members  the  limbs  subsequently  suffered.  In  cases  in  which 
the  wasting  spreads,  years  may  intervene  before  the  extension  takes 
place.    In  one  case,  for  instance,  the  right  arm  became  affected  at 

{'  Riv.  Clin.,'  June,  1887),  and  twelve  years  later  hud  involved  also  the  legs.  The 
pritient's  mother,  two  brothers,  sister,  and  maternal  uncle  suffered  in  the  same  way 
at  the  same  age. 


SIMPLE    IDIOPATHIC    MUSCULAR   ATROPHY. 


589 


nineteen,  the  left  at  twenty-five,  and  tlie  legs  at  thirty.  In  another  the 
arms  began  to  waste  at  thirty-five,  power  of  standing  was  only  lost  at 
fifty-five,  and  the  patient  lived  till  seventy-five.    Even  when  the  malady 


I'ra.  1fi9. 


Fro,  170. 


Fro. 171 


Figs.  169  to  171. — Idiopathic  muscular  atrophy  affecting  the  face.  Fig.  I(i9 
shows  the  wasting  of  the  deltoid  upper  arm  muscles  and  supinator  lontrus 
(while  the  forearm  muscles  are  not  wasted),  and  the  rotation  of  the  scapula 
from  the  loss  of  the  trapezius  and  rhomboids.  Fig.  170  shows  the  habitual 
appearance  of  the  face,  and  Fig.  171  the  greatest  possible  movement  of  tlie 
facial  muscles  in  closing  the  eyes  and  smiling,  and  also  the  wasting  of  the 
deltoids  and  pectorals.  The  general  distribution  of  the  atrophy  and  projec- 
tion of  the  scapnlse  are  seen  in  Fiir.  172.* 


•  The  patient  was  a  lad  sixteen  years  of  age.  The  following  is  an  abstract  of 
the  notes  of  his  case.  In  his  family  no  history  of  any  similar  affection  can  be 
ascertained.  There  is  conclusive  evidence  that  he  had  a  hard  chancre  at  the  age 
of  three,  followed  by  secondary  symptoms.  The  muscular  wasting  began  gradually 
during  childhood;  even  when  very  young  there  was  little  movement  in  the  face,  and 
that  ceased  at  the  age  of  six  or  seven.  Walking  became  difficult  about  three  years 
ago.  The  face  is  almost  motionless:  an  effort  to  smile  causes  only  a  just  perceptible 
movement  of  the  left  cheek,  the  amount  of  which  is  fairly  indicated  by  the  difference 
between  Pigs.  170  and  171.  It  is  accompanied  by  a  distinct  movement  of  the  ears. 
The  lips  are  full  and  can  be  brought  together,  but  not  shortened.  The  eyelids  cannot 
be  made  to  meet,  even  with  a  strong  effort,  being  then  |  inch  apart.  Forehead 
absolutely  motionless.  Eyeballs  prominent,  movements  normal.  Masseters,  tongue, 
pharynx,  and  larynx  unaffected.  In  the  neck  the  sterno-mastoids  are  very  small  and 
feeble,  the  cmo-hyoids  large  and  strong.     The  following  muscles  seem  quite  gone^ 


590 


SPINAL    OORD. 


Pra.  T 


begins  in  childhood  its  progress  is  sometimes  very  slow.  Thus  in  one 
case  the  affection  of  the  face  was  first  noticed  at  five,  at  twelve  the 
arms  began  to  suffer,  and  a  few  years  later 
the  flexors  of  the  hip  became  weak,  but  the 
patient  was  still  able  to  walk  at  the  age  of 
forty.  The  disease  may  increase  until  deve- 
lopment is  over  and  then  remain  stationary, 
as  in  a  case  in  which,  at  forty-four,  there 
had  been  no  change  since  the  age  of  twenty. 
The  patient's  daughter  was  more  severely 
affected.*  On  the  other  hand,  the  progress 
of  the  disease  may  be  more  rapid  and  uni- 
form, and  the  atrophy  may  reach  its  widest 
distribution  in  eight  or  ten  years.  In  most 
cases,  even  of  severe  type,  the  wasting  docs 
not  become  universal,  but  remains  limited  to 
the  muscles  mentioned  above,  but  occasionally 
hardly  any  muscles  of  the  body  may  escape. 

The  duration  of  the  disease  varies  from  ten 
to  fifty  years.  Death  has  never  occurred  as 
the  direct  result  of  the  malady.  In  the  cases 
of  most  severe  degree  and  rapid  course  the 
patient  has  usually  died  from  phthisis,  pro- 
bably related  to  the  deficient  breathing  power, 
just  as  in  the  sufferers  from  pseudo-hypertro- 
phic  paralysis.  In  most  cases,  however,  death 
has  been  due  to  other  maladies,  and  has  not 
been  in  any  degree  the  consequence  of  the  muscular  disease. 

Pathological  Anatomy. — In  general,  the  condition  of  the  mus- 
cular fibres  resembles  that  met  with  in  pseudo-hypertrophic  para- 
lysis, the  increase  of  interstitial  tissue  being  wanting  except  in  those 

trapezius  (except,  perhaps,  a  little  of  the  middle  part  of  the  right),  rhomboids, 
deltoid,  peotorales,  latissimus  dorsi,  serrati,  biceps,  bracliialis,  triceps,  supinator 
longus.  The  lev.  ang.  scapulse,  supra-  and  infra-spinatus  are  unaffected;  the  latter 
is  large,  but  probably  only  hypertrophied  from  over-use.  The  supinator  longus  is 
fei  hie  on  the  left,  powerless  on  the  right  side.  The  extensors  of  the  phalanges  of 
the  thumb  are  lost  on  the  left  side,  and  the  extensor  carpi  radialis  on  the  right.  All 
the  other  forearm  and  hand  muscles  appear  to  be  unaflfected.  The  erectors  of  the 
spine  are  small  and  feeble;  intercostals  strong,  diaphragm  powerless,  abdomin;il 
muscles  weak.  In  the  legs  the  glutei  and  flexors  of  the  knees  seem  normal,  the  flexors 
of  I  he  hips  weak,  extensors  of  the  knees  small  and  feeble,  muscles  of  the  lower  leg 
rather  small  and  feeble,  but  only  the  peronei  are  powerless,  right-angled  contracture 
of  ankle-joint  with  tendency  to  varus.  Knee-jerk  absent.  Circumference  (in 
inches)  of  upper  arms  (middle),  R.  5-^,  L.  5|-;  of  forearm  (maximum),  each  8;  of 
tliighs  (minimum  2J  inches  above  patella),  fl.  10^,  L.  9^;  calves  (maximum), 
R.  lOf,  L.  11.  Electric  irritability  much  lowered  in  all  aff'ected  muscles  to  each 
current;  in  most,  no  contraction  can  be  obtained.  Sensation,  the  sphincters,  and 
the  viscernl  functions  are  unaffected. 
•  Landouzy  and  Dejerine,  loc.  cit. 


SIMPLE    IDIOPATHIC    MUSCULAR    ATROPHY.  591 

cases  that  really  belong  to  this  form,  but  in  wliicli  the  characteristic 
enlargement  is  absent,  because  no  fat-cells  are  deyeloped  in  the  tissue. 
A  multiplication  of  nuclei  is,  however,  sometimes  seen.  The  chief 
change  is  sometimes  a  simple  narrowing  of  the  fibres,  "vith  an  ultimate 
disappearance  of  the  transverse  striation.  Degenerative  changes  are 
frequently  met  with,  indistinct  striation  or  granular,  fatty,  or  "  waxy  " 
transformation  being  seen  here  and  there,  alike  in  narrowed  or 
enlarged  fibres,  and  in  those  that  retain  their  normal  calibre ;  occa- 
sionally fibres  present  longitudinal  striation,  fissuring,  or  vacuolation  ; 
but  in  other  cases,  or  in  some  muscles  (especially  in  the  "juvenile" 
form),  a  remarkable  increase  has  been  found  in  the  size  of  fibres  in 
excised  fragments,*  even  of  muscles  that  are  below  the  normal  size. 
It  seems  to  show  definite  pathological  enlargement. 

The  statements  made  regarding  the  spinal  cord  in  the  pseudo- 
hypertrophic form  are  strictly  applicable  also  to  this  variety.  It  is 
normal  as  a  rule,  to  which  exceptions  are  so  rare  that  they  can  have 
no  relation  to  the  malady,  save  that  of  secondary  indirect  consequences. 
The  nerves  also  are  normal,  if  the  cases  are  eliminated  in  which  the 
symptoms  indicate  the  peroneal  form. 

Thus  the  facts  ascertained  regarding  this  type  do  not  suggest  any 
special  addition  to  the  general  conclusions  intimated  in  the  introduc- 
tory paragraphs.  The  affection  appears  to  be  the  result  of  a  primary 
developmental  tendency,  involving  the  muscular  tissue  only,  and 
cannot  be  regarded  as  a  secondary  result  of  the  interstitial  changes, 
except  in  a  trifling,  occasional,  and  unimportant  degree.  Still  less  is 
it  a  consequence  of  changes  in  the  spinal  cord.  If  it  is  in  any  measure 
the  result  of  failure  in  the  nutrition  of  the  terminal  structures  of  the 
nerve-fibres  (by  which  the  nervous  and  muscular  tissues  are  connected, 
and  which  is  perhaps  to  be  regarded  as  intermediate  between  the  two) , 
the  fact  has  yet  to  be  demonstrated,  and  its  significance  ascertained. 

Diagnosis. — The  two  most  important  diagnostic  indications  are  the 
affection  of  more  than  one  member  of  the  same  family,  and  the  onset 
of  the  disease  before  adult  life  is  reached.  The  former  is  practically 
conclusive ;  and  the  latter  should  always  suggest  the  probable  idiopathic 
nature  of  the  case.  In  isolated  cases  the  diagnostic  difficulty  is  much 
greater,  and  no  indication  is  actually  conclusive,  except,  perhaps,  the 
affection  of  the  face.  Commencement  during  childhood  is  also  of  great 
weight.  It  is  most  unlikely  that  progressive  muscular  atrophy, 
begiuning  under  ten,  is  of  spinal  origin,  but  I  have  known  such 
atrophy,  certainly  spinal,  to  commence  at  the  age  of  fourteen.f  The 
peculiar  affection  of  the  zygomatic  muscles  is  very  characteristic,  but 
we  cannot  yet  say  that  it  is  pathognomonic  (see  note  on  p.  587),  and 
the  affection  of  the  lips  must  be  carefully  distinguished  from  that  due 
to  the  bulbar  palsy  so  commonly  associated  with  spinal  atrophy.     Of 

*  See  especially  Schulze,  '  Ueber  deu  mit  Hypertrophie  verbundenen  Maskel* 
fichwund.'  Wiesbaden,  1886,  and  Hitzig,  loc.  cit. 
t  See,  however,  p.  597. 


592  SPINAL   CORD. 

tlie  distribution  of  the  -wasting,  that  of  the  latissimus  and  lower  half  of 
the  pectorahs,  and  the  escape  of  the  hand  muscles  are  the  most  im- 
portant characteristics,  although  they  are  not  by  themselves  con- 
clusire,  since  they  are  sometimes  met  with  in  spinal  atrophy. 

But  the  most  important  distinctions  between  spinal  and  idiopathic 
atrophy,  whenever  they  are  available,  depend  upon  the  symptoms  of 
a  lesion  of  the  cord,  or  of  the  bulbar  nuclei.  Especially  significant 
is  the  excess  of  the  knee-jerk  which  results  from  lateral  sclerosis,  a 
conclusive  indication  of  spinal  disease,  rendering  the  spinal  origin  of 
the  atrophy  all  but  certain.  The  distinction  from  the  pseudo-hyper- 
trophic  form  has  been  already  considered ;  that  from  the  peroneal  type 
will  be  presently  mentioned. 

Prognosis. — The  extreme  variations  in  the  course  of  the  disease 
render  the  prognosis  in  any  individual  case  both  uncertain  and  difficult 
to  formulate.  Speaking  generally,  chronicity  favours  arrest,  and  the 
later  the  symptoms  appear  the  slower  will  be  their  progress.  The 
prognosis  is  distinctly  less  grave  in  the  simple  atrophy  than  in  the 
"juvenile  "  atrophic  variety  of  the  pseudo-hypertrophic  disease,  and  in 
a  considerable  number  of  cases,  perhaps  one  half,  the  malady  has  not 
appeared  to  shorten  life.  Even  in  cases  that  begin  during  youth  it  is 
therefore  possible  that  the  patient  may  reach  old  age.  It  seems  also 
to  be  better  in  the  cases  that  do  not  involve  the  facial  muscles. 

Teeatmbnt. — It  is  not  yet  clear  that  idiopathic  muscular  atrophy 
can  be  influenced  by  treatment  in  any  considerable  degree.  Most 
published  records  of  cases  are  almost  silent  on  the  subject,  and  the 
disease  is  so  rare  that  few  individuals  have  an  opportunity  of  forming 
an  opinion.  It  might  be  assumed  that  the  essential  cause  of  the 
disease,  a  congenital  tendency,  withdraws  it  from  the  range  of  thera- 
peutics, but  the  course  of  the  malady  is  very  different  from  that  of 
some  other  diseases  which  own  a  similar  cause.  The  extreme  variations 
in  the  date  of  onset,  the  fact  that  the  disease  may  not  be  manifested 
until  late  in  life,  and  the  long  period  that  may  intervene  between  its 
onset  and  extension,  all  suggest  that  other  influences  co-operate  with 
the  congenital  tendency  in  determining  the  development  of  the  malady. 
It  is  quite  possible,  therefore,  that  the  first  inference  from  the  history 
of  the  disease  may  not  be  altogether  correct,  although  it  must  be 
admitted  that  we  have  not  as  yet  any  evidence  that  the  disease  is 
susceptible  of  influence  from  drugs.  Electrical  treatment  and  mass- 
age have  been  thought  to  do  good  and  even  to  produce  arrest  (Erb), 
but  the  variable  tendency  of  the  malady  renders  the  evidence  of  arrest 
insufficient.  If  voluntary  exercise  is  practicable,  this  is  a  far  more 
efficient  stimulus  to  muscular  growth  than  any  electrical  application, 
and  it  is  probable  that  such  exercise,  carefully  persevered  in,  may  do 
something  to  prevent  the  occurrence  of  the  malady  in  those  pre- 
disposed to  it,  and  even  to  retard  its  progress  in  those  who  are 
already  attacked.  In  this  connection  it  is  noteworthy  that  very  few 
of  the  sufferers  have  been  engaged  in  occupations  that  involve  active 


PERONEAL   TYPE  OP  AMYOTROPHY.  593 

mnscular  exertion.  Over-exertion  should,  however,  be  carefully 
avoided.  Tlie  general  health  should  be  attended  to,  and  any  defect 
removed  as  speedily  as  possible,  both  in  those  who  suffer  and  in  those 
who  are  related  to  sufferers,  since,  as  we  have  seen,  depressing 
influences  may  apparently  excite  the  development  of  the  disease,  and 
it  is  therefore  reasonable  to  suppose  that  they  may  accelerate  its 
course,  and  lessen  any  tendency  there  may  be  for  the  morbid  process 
to  become  stationary. 


The  Pbeoneaii  Type  of  Famtlt  Amtoteopht. 
{Neuritic  Muscular  Atrophy.') 

The  name  "  peroneal  type  "  has  become  current  as  the  most  con- 
venient designation  for  the  variety  of  muscular  atrophy,  occurring  in 
early  life,  to  which  it  was  applied  by  Dr.  Howard  Tooth  in  a  Thesis 
published  in  1886.*  This  was  the  means  of  calling  general  attention 
to  the  form,  of  which,  however,  an  account  had  been  published 
shortly  before  by  Charcot  and  Marie,t  and  many  cases  had  been  pre- 
viously described,  in  most  instances  without  recognition  of  their  special 
character.  J  The  cases  of  this  form  present  certain  resemblances  to 
the  idiopathic  atrophies  just  described — resemblances  sufficiently 
important  to  have  led  to  the  description  of  these  cases  in  association 
with  the  primary  myopathies,  embracing,  as  they  do,  the  age  of  the 
sufferers,  the  occurrence  of  the  disease  in  members  of  the  same  family, 
its  gradual  onset,  and  its  very  slow  but  progressive  course.  Yet  this 
position  must  be  regarded  as  provisional  only,  and  probably  erroneous. 
The  distribution  of  the  wasting  involves  a  constant  difference  from 
the  idiopathic  musc\ilar  affections,  and  some  of  the  cases,  otherwise 
inseparable  from  the  rest,  present  features  that  indicate  neuritis.  The 
condition  may  have,  as  an  antecedent,  an  acute  specific  disease — a  circum- 
stance that  has  the  same  significance.  The  facts  ascertained  con- 
cerning this  form  must,  therefore,  be  carefully  excluded  from  our 
generalisation  regarding  idiopathic  atrophy. 

In  this  disease,  males  suffer  about  twice  as  frequently  as  females. 
It  generally  begins  in  the  second  half  of  childhood,  and  very  seldom 
after  twenty,  although  cases  have  been  described  beginning  as  late 
as  forty.  It  is  occasionally  hereditary,  and  still  more  frequently 
collaterals  suffer,  several  brothers  and  sisters  being  affected.  The 
wasting  is  first  apparent  in  the  extensor  longus  hallucis,  or  extensor 
communis  digitorum,  or  in  the  peronei  muscles.  According  to  Tooth 
it  very  often  begins  in  the  latter,  and  occasionally  m  the  gastrocnemius, 
but  it  is  probable  that  it  occurs  simultaneously  or  ev^en  earlier  in  the 

*  *  The  Peroneal  Type  of  Progressive  Muscular  Atrophy,'  London,  Lewis,  1886. 
t  •  Revue  de  Med.,'  1886,  p.  97. 

J  By  Friedreich,  Eichliorst,  Oppenheimer,  Ormerod,  and  others.  A  table  of 
thirty  collected  cases  (some  doubtful)  is  given  by  Tooth. 

VOL.  I.  38 


594  SPINAL    COED 

small  muscles  of  tlie  foot,  where  it  readily  escapes  observation.  Such 
early  atrophy  ia  the  feet  has,  iadeed,  been  actually  found  in  some  cases 
that  came  under  observation  at  an  early  stage.*  The  calf  muscles 
suffer  subsequently,  and  still  later  those  of  the  thigh,  especially  the 
vastus  internus.  The  unequal  affection  of  the  muscles  of  the  leg 
causes  the  early  development  of  club-foot,  which,  especially  in  children, 
becomes  a  characteristic  symptom.f  The  arms  are  only  invaded,  in 
most  cases,  some  years  after  the  legs,  so  slow  is  the  course  of  the 
disease.  The  intrinsic  muscles  of  the  hands  (thenar,  hypothenar, 
and  interosseal  muscles)  are  first  attacked,  and  subsequently  those  of 
the  forearm :  sometimes  the  extensors  and  sometimes  the  flexors  suffer 
first  and  most ;  while  the  supinator  longus  remains  normal,  and  so  do 
the  muscles  of  the  shoulder,  neck,  and  back.  The  affection,  however, 
does  not  necessarily  commence  in  the  lower  limbs,  and  the  face  may 
be  affected  (Hoffmann).  The  atrophy  is  often  symmetrical,  but  occa- 
sionally the  muscles  on  one  side  waste  first  a,nd  most.  The  claw-like 
deformity  of  the  fingers  may  result  from  the  affection  of  the  intrinsic 
muscles  of  the  hand.  This  is  so  rare  in  early  life  from  any  other 
cause  that  its  significance  is  considerable,  and  almost  distinctive  when 
combined  with  the  deformity  of  the  feet  just  mentioned.  The  ca,se 
shown  in  Fig.  173  was  probably  an  example  of  this  type.  J 


Fia.  173. — Advanced  muscular  atrophy,  probably  of  the  "peroneal  type." 
(Drawn  by  Dr.  Spencer,  from  a  photograph.) 

The  affected  muscles  (especially  in  the  hand)  occasionally  present 
slight  fibrillation.     Their  electric  irritability  is  diminished  sooner,  and 

•  Hoffmann,  'Arch.  f.  Psych.,'  xx,  p.  660 j  Joffroy,  '  Gaz.  Hebd.,'  1886,  No.  18. 

t  See  Sachs,  •  Brain,'  Winter  part,  1889. 

J  lb  was  given  in  the  first  edition  as  an  example  of  simple  atrophy.  The  patient 
was  a  man  aged  twenty-seven  at  the  time  of  his  death  (in  University  College 
Hospital).  No  history  of  any  analogous  case  in  his  family  could  be  ascertained. 
The  affection  began  at  the  age  of  fourteen,  when  his  feet  began  to  turn  in  so  that 
he  walked  on  the  outer  side  of  the  foot,  and  soon  he  noticed  graduiil  wasting  of 
the  legs,  which  slowly  progressed,  and  about  the  age  of  twenty-four  the  arms  also 
began  to  suffer.  "When  first  seen,  a  few  months  before  his  death,  the  muscular 
atrophy  was  universal,  and  the  subcutaneous  fat  had  also  disappeared.  Even  the 
hands  were  greatly  wasted;  there  was  a  hollow  in  the  position  of  the  thenar 
eminence,  just  as  in  progressive  muscular  atrophy,  and  the  interosseal  muscles  were 
also  greatly  wasted.  There  was  slight  fibrillation.  The  intercostals  were  paralysed. 
The  wasting  of  the  legs  was  extreme;  the  maximum  circumference  of  the  calf  wai 


PERONEAL  TYPE  OF  AMYOTROPHY.  595 

in  greater  degree,  than  in  tlie  ordinary  idiopathic  atrophy  .while  a  stiU 
greater  difference  is  presented  by  the  fact  that  f aradic  irritability  may 
early  become  extinct,  and  that  there  may  be  a  distinct  reaction  of 
degeneration.  In  connection  with  this  should  be  noted  the  important 
fact  that  cutaneous  sensibility,  though  often  nonnal,  is  sometimes 
impaired  or  lost,  especially  over  the  region  in  which  the  atrophy  is 
greatest,  or  upon  the  soles  of  the  feet.  Pains  occur  in  some  cases, 
and  so  also  do  spontaneous  spasmodic  contractions,  especially  in  the 
muscles  of  the  thigh.  The  muscle-reflex  action  is  lessened  or  lost  in 
the  affected  regions,  but  there  may  be  extensive  atrophy  below  the 
knees,  without  loss  of  the  knee-jerk,  if  the  thigh  muscles  are  pre- 
served. Cutaneous  reflex  action  presents  considerable  variations,  aaid 
is  preserved  more  often  than  would  be  expected.  Yaso-motor  dis- 
turbance sometimes  occurs  (Sachs). 

The  nature  of  these  cases  has  still  to  be  demonstrated.  Their  very 
slow  course,  the  time  of  life  at  which  they  begin,  and  the  affection  of 
several  members  of  the  same  family,  are  features  analogous  to  the 
idiopathic  atrophy ;  but  a  marked  contrast  to  this  is  presented  by  the 
tendency  of  the  malady  to  succeed  an  acute  specific  disease,  measles, 
and  this  also  may  appear  as  a  family  tendency.  This  feature  re- 
sembles the  causation  of  multiple  neuritis,  and  the  electrical  reaction 
of  degeneration  is  conclusive  proof  of  preponderant  changes  in  the 
motor  nerves,  while  anaesthesia  demonstrates  that,  in  some  cases,  the 
sensory  fibres  are  also  affected.  In  such  cases  a  peripheral  neuritis, 
motor  or  total,  of  extremely  chronic  course  and  peculiar  origin,  must 
be  recognised  as  the  only  possible  lesion,  and  it  was  actually  found, 
in  one  case  by  Friedreich,  and  in  another  by  Gombault.  Dubreiulh* 
also,  in  a  case  which  he  examined,  found  that  there  were  inflammatory 
changes  in  the  nerves,  the  spinal  cord  being  normal.  If  this  is 
the  cause  of  the  symptoms  in  some  instances,  the  question  arises 
whether  it  is  not  the  lesion  in  all  the  cases  of  this  peroneal  type. 
Such  a  lesion  is  conceivable;  the  degenerative  form  of  neuritis 
presents  every  degree  of  chronicity,  and  may  be  limited  to  the  motor 
fibres  and  to  certain  muscles.  This  pathology  was  advocated  as  the 
probable  one  by  Tooth,  who  pointed  out  that  it  would  also  enable  us 
to  understand  the  occurrence  of  fibrillation,  which  is  extremely  rare 
in  the  idiopathic  atrophies.  It  is,  indeed,  asserted  by  Hoffmann  that 
not  only  are  the  peripheral  nerve-fibres  the  seat  of  a  primary  degene- 
ration, but  that  this  change  ascends  the  nerves  to  the  anterior  and 
posterior  roots,  and  involves,  in  a  secondary  manner,  the  elements  of 

only  seven  inches;  the  feet  were  everted,  the  sole  hollowed,  and  the  toes  flexed. 
The  patient  died  from  diarrhoea.  The  muscles  were  foaud  to  be  small  and  pale. 
The  chief  microscopical  change  was  intense  granular  and  fatty  degeneration  of  the 
fibres.  A  very  few  normal  fibres  were  seen  in  some  muscles.  There  was  no  increase 
ot  interstitial  tissue.  Microscopical  examination  revealed  no  morbid  changes  in  the 
spinal  cord.  The  nerves,  unfortunately,  were  not  examined. 
*  •  Rev.  de  Med.,'  1890;  *Neur.  Cent.,*  1891. 


696  SPINAL   CORD. 

the  spiual  cord,  especially  the  fibres  of  the  posterior  median  columna 
(which  continue  the  path  from  the  muscles)  and  the  motor  cells  of 
the  anterior  cornua.  But  we  do  not  know  how  far  the  premature 
failure  of  nutrition  is  a  secondary  process,  transmitted  from  below, . 
or  how  far  it  is  the  result  of  a  defect  in  vital  energy,  shared  by  the 
central  as  well  as  the  peripheral  nerve-elements. 

I'he  intimate  relation  between  the  motor  nerves  and  the  nniscular 
fibres  should  also  be  kept  in  view  in  this  connection.  There  may 
be  a  morbid  tendency  in  the  nerves  similar  to  that  of  the  muscles, 
including  a  liability  to  early  slow  degeneration,  analogous  to  that 
which  the  muscles  present,  and  equally  prone  to  occur  in  several 
members  of  a  family.  iut  it  is  desirable  that  the  neuritic  nature 
of  the  affection  shoula  oe  placed  on  a  broader  basis  of  ascertained 
fact  before  it  is  -liliowed  expression  in  the  name  given  to  the  disease. 

It  should  also  be  remembered  as  possible  that  the  atrophy  may  be 
idiopathic  in  some  cases,  while  in  others  it  may  be  secondary  to  such  a 
neuritis  as  above  mentioned.  There  is  nothing  difficult,  or  even 
anomalous,  in  the  conception  of  a  congenital  tendency  to  premature 
failure  of  nutrition  in  the  peripheral  nerve-fibres,  like  that  which,  in 
the  posterior  column  and  other  fibres,  seems  to  underlie  "  hereditary 
ataxy,"  or  which,  on  the  other  hand,  in  the  muscles  is  the  apparent 
cause  of  idiopathic  atrophy.  Further,  if  the  lesion  is  really  neuritic,  we 
can  understand  that  an  acute  specific  disease  like  measles  should  occa- 
sionally be  its  excitant,  although  its  occurrence  in  famihes  shows  that 
a  congenital  tendency  must  be  called  into  activity  by  the  virus,  and  the 
cases  must  be  distinguished  from  those  in  which  a  pure  neuritis  is  in- 
duced by  the  influence  of  the  poison  of  the  specific  disease.  An  acute 
specific  disease,  the  virus  of  which  is  known  to  be  capable  of  inducing 
the  degeneration  of  "  parenchymatous  "  neuritis,  may  readily  excite  this 
state  in  each  of  several  members  of  the  same  family  if  a  congenital 
tendency  to  it  exists  in  them.  It  may  readily  evoke  that  which  it 
could,  even  alone,  produce.  According  to  this  theory,  a  tendency  to 
nerve  degeneration,  acting  alone,  may  lead  to  the  early  failure  of 
nutrition  of  the  nerves,  while  the  wasting  of  the  muscular  fibres 
may  be  either  a  consequence  or  a  concomitant,  in  part  or  altogether. 
Thus  the  muscles  may  atrophy  because  the  nerves  have  degenerated, 
or  because  they  also  have  a  tendency  to  atrophy,  or  from  both  causes 
combined.  Nerve  and  muscle  may  alike  share  the  congenital  imper- 
fection in  some  cases,  while  in  others  its  primary  incidence  is  on  the 
nerves  alone,  the  muscles  suffering  in  consequence  of  the  changes  in 
the  nerves.  The  congenital  association  may  sometimes  be  even  more 
extensive,  and  a  tendency  to  the  early  failure  of  structures  in  the 
central  nervous  system  may  be  conjoined,  giving  rise  to  cases  of  com- 
plex aspect  and  character. 

It  is  especially  important  to  note,  in  this  connection,  the  occurrence 
of  cases  that  occupy  an  intermediate  position,  and  combine  the 
characters  of  this  and  the  myopathic  types.     Such  combined  cases  are 


MUSCULAR  ATEOPHY.  597 

usually  partial  only,  but  sucli  partial  cases,  when  combined,  ultimately 
cover  the  whole  intermediate  ground.  For  instance,  the  condition  of 
the  legs  in  the  "  peroneal  type  "  has  co-existed  with  that  of  the  arms 
in  the  "juvenile  form"  in  several  recorded  cases.* 

Thus  the  possibility  must  be  recognised  that  the  cases  of  this  type 
vaiy,  even  more  than  their  characters  suggest,  in  the  degree  in  which 
the  morbid  tendency  acts  upon  nerve  or  muscle,  or  both,  as  well  as 
the  degree  in  which  its  activity  is  spontaneous  or  induced.  Hence  the 
cases  may  here  be  only  partly  out  of  place — some,  perhaps,  not  at  aU ; 
and  their  position,  as  forms  of  "  neuritis,"  might  be  equally  open  to 
exception. 

The  progressive  character  of  the  cases  of  this  type  is  commonly  pro- 
nounced ;  and,  even  if  slow,  it  renders  the  prognosis  gloomy,  and  the 
scope  for  treatment  small.  So  far  as  anything  can  be  done,  it  is  by  the 
measures  already  mentioned  in  the  account  of  the  treatment  of  the 
maladies  to  which  the  cases  approximate,  the  primary  diseases  of  the 
nerves  on  the  one  hand,  and  of  the  muscles  on  the  other. 


Family  Poem  op  MusctriiAE  Atrophy  in  Childeeit  with  Spinai. 

Lesion. 

The  above  seems  to  be  the  most  suitable  title  for  a  series  of  cases 
described  in  recent  years  by  Werdnig  f  and  Hoffmann  J  in  Grermany, 
and  Bruce  and  John  Thomson  §  in  this  country.  The  condition  is 
marked  by  the  occurrence  of  similar  atrophy  in  more  than  one 
member  of  the  family,  although  in  Bruce  and  Thomson's  case  the 
condition  was  an  isolated  one.  It  is  characterised  by  the  onset  of 
weakness,  especially  in  the  hip  and  back  muscles,  about  the  age  of 
from  seven  to  twelve  months,  in  children  previously  healthy.  The 
weakness  gradually  impairs  and  finally  abolishes  the  power  of  walking, 
and  similar  weakness  usually  attacks  the  upper  limbs  as  well  as  the 
legs,  and  even  the  small  muscles  of  the  hand  may  be  affected.  The 
condition  is  one  of  marked  atrophy  without  sensory  impairment, 
although  in  one  case  there  was  a  curious  insensibility  to  the  pains  of 
faradic  stimulation  of  the  affected  muscles.  In  many  of  the  muscles 
the  reaction  of  degeneration  may  be  present,  and  occasionally  bulbar 
symptoms  are  superadded.  1'he  condition  culminates  in  one  of 
extreme  helplessness,  with  marked  wasting  of  muscles  without  any 
fibrillary  twitching,  although  death  did  not  take  place  in  some  cases 
until  the  age  of  six  was  attained.  No  hypertrophy  has  been  noted  in 
any  case. 

*  JE.g.  by  Hoffmann,  'Berlin,  klin.  Wochenschr.,'  1887,  No.  22;  and  Eisenlohr, 
*Neur.  Centralbl.,'  1889,  p.  565. 
t  'Arch.  f.  Psych  ,'  1891  and  1894. 
X  •  Deutsch.  Ztsch.  fiir  Nervenheilk.,'  1893  and  1897, 
§  'Edin.  Hosp.  Reports,'  1893. 


598  SPINAL  CORD. 

The  conditions  found  after  death  were  marked  atrophy  of  nmscular 
fibres,  although  occasionally  some  fibres  were  hypertrophied.  Atrophy 
also  of  nerves  and  anterior  roots  was  found,  and  wasting  of  the  cells  in 
the  anterior  horns.  The  latter  was  distinct,  and  this,  with  the  absence 
of  any  hypertrophy  at  any  time  (although  one  child  had  been  noted  as 
very  fat  which  afterwards  became  much  wasted),  marks  off  the  cases 
distinctly  from  those  of  pure  muscular  dystrophy.  The  type  possibly, 
like  the  peroneal,  forms  a  connecting  link  between  cases  of  spinal  pro- 
gressive muscular  atrophy  and  those  of  true  myopathy  without  any 
spinal  lesion.  The  presence  of  definite  changes  in  the  cells  of  the 
anterior  horn  and  the  anterior  nerve-roots  connects  the  cases  with 
the  former,  while  a  connection  with  the  latter  is  formed  by  the  simple 
wasting  vrith  occasional  hypertrophy  of  isolated  muscular  fibres,  and 
by  the  occurrence  of  the  disease  in  more  than  one  member  of  a  family. 

Diagnosis. — The  age  at  which  the  disease  occurs,  the  absence  of 
fibrillary  twitching,  and  the  early  suppression  of  the  reflexes,  as  well 
as  the  frequent  occurrence  of  more  than  one  case  in  the  same  family, 
will  suflEciently  distinguish  these  cases  from  ordinary  progressive 
muscular  atrophy.  On  the  other  hand,  the  absence  of  any  hyper- 
trophy, the  affection  of  the  small  muscles  of  the  hand,  and  the 
occasional  presence  of  bulbar  symptoms  will  mark  them  off  from 
cases  of  true  muscular  dystrophy.  In  every  case  which  comes  under 
notice  hypertrophy  in  some  muscles,  especially  the  infraspinati,  should 
be  carefully  looked  for,  as  it  may  turn  out  that  the  cases  are  more 
closely  connected  with  myopathic  cases  than  is  as  yet  apparent  from 
the  evidence. 

Pbognosis. — This  seems  to  be  extremely  bad ;  the  cases  go  on  from 
bad  to  worse,  the  weakness  becomes  greater,  the  atrophy  more  pro- 
nounced, until  death  occurs.  But,  as  has  been  stated  above,  this  has 
not  unfrequently  been  delayed  as  late  as  the  sixth  year. 

Treatment. — The  nature  of  such  cases,  indicating  as  they  do  an 
inherited  tendency  to  early  death  in  the  muscular  fibres  and  their 
correlated  nervous  structures,  precludes  the  hope  of  successful  treat- 
ment. The  administration  of  strychnia  hypodermically  is  indicated, 
although  the  age  of  the  patient  renders  extreme  care  necessary  in  the 
choice  of  doses.  It  is  also  possible  that  galvanism,  combined  with 
massage  and  passive  exercise,  might  have  a  beneficial  effect.  And,  of 
course,  care  must  be  taken  to  ensure  wholesome  and  judicious  feeding 
and  satisfactory  hygienic  conditions. 


Periodic  Family  Paralysis. 

Under  this  name  Goldflam  *  has  described  a  condition  of  which 
examples  had  been  previously  published  by  Westphal,  Cousot,  and 
Schachnovdtsch.  It  is  characterised  by  recurrent  paralysis  commencing 

•  Berlin  Congress,  1890;  'Neur.  Cent.,'  1890,  p.  638. 


MUSCULAR  HYPERTROPHY.  599 

usually  in  the  legs,  involving  also  the  arms,  and  passing  off  in  the 
reverse  order  after  a  period  varying  from  twenty-four  hours  to  three 
days.  During  the  attack  of  paralysis  the  patient  is  sleepy,  but  there 
is  no  impairment  of  consciousness,  sensibility  is  retained,  and  the 
paralysis  is  flaccid,  with  diminished  knee-jerks  and  loss  of  plantar 
reflexes.  Speech  and  swallowing  are  not  interfered  with.  In  Gold- 
flam's  case  there  was  great  diminution  of  faradic  irritability  in  the 
upper  limbs,  and  complete  loss  in  the  lower  limbs.  After  the  attack 
had  passed  off  the  nerves  in  both  arms  and  legs  reacted  normally. 
Eleven  members  of  the  family  suffered  from  similar  attacks  of 
paralysis.  They  began  between  fiiteen  and  twenty,  and  increased  in 
number  as  age  advanced,  although  the  individual  attacks  did  not  last 
so  long.  In  the  patient  whose  case  is  particularly  described  there  were 
only  two  attacks  in  the  first  year,  but  after  three  years  they  occurred 
every  fortnight. 

The  condition  as  regards  etiology  is  obscure.  Groldflam's  opinion  is 
that  it  is  the  result  of  auto-intoxication — a  view  which  is  favoured  by 
the  fact  that  m  his  patient  obstinate  constipation  persisted  during 
each  attack  of  paralysis. 

Apparently  the  disease  does  not  in  any  way  tend  to  shorten  Hfe,  but 
treatment  is  of  little  if  any  effect. 

Mttsculab  Htpeeteopht. 

The  occurrence  of  a  true  hypertrophy  of  muscles,  as  a  condition  of 
disease,  is  exceedingly  rare.  In  most  cases  in  which  the  muscles  are 
enlarged,  the  increase  in  size  is  due  to  a  growth  of  interstitial  tissue, 
fatty  or  fibrous,  such  as  has  been  described  in  the  chapter  on  pseudo- 
hypertrophic paralysis.  We  have  seen  that  muscular  fibres  larger 
than  normal  have  been  described  in  that  disease,  and  also  in  some 
cases  of  simple  atrophy.  When  this  increase  in  size  has  been  met 
with  only  in  excised  fragments,  it  is  possible  that  the  condition  may 
have  been  due  to  a  vital  contraction  excited  by  the  process  of  excision, 
and  such  evidence  of  hypertrophy  is  certainly  inadequate  ;*  only  when. 

*  Since  the  above  was  written,  the  doubt  there  expressed  has  been  confirmed  by 
an  observation  made  for  me  by  Dr.  H.  R.  Spencer.  A  fragment  of  the  gastrocnemius 
of  an  amputated  leg  was  excised  immediately  after  the  amputation,  and  the  fibres 
were  compared  with  those  of  the  muscle  twenty-one  hours  later.  In  order  to 
ascertain  more  definitely  whether  a  vital  contraction  persisted  as  an  apparent 
increase  in  size,  a  part  of  the  excised  fragment  was  separated  and  faradised.  The 
average  size  of  the  fibres  in  the  muscle  was  i^^  inch,  in  the  fragment  excised 
g-i-g-  inch,  in  the  fragment  faradised  -^^j-  inch.  Since  the  division  of  the  nerve 
during  the  amputation  may  have  caused  some  contraction,  it  is  possible  that  the 
dilference  in  size  produced  by  excision  may  be  even  greater  than  these  figures 
represent.  [I  leave  the  above  note  unchanged  because,  since  it  appeared  in  the  first 
edition,  the  observation  and  doubt  have  been  fully  confirmed  by  various  observers, 
e.g.  by  Oppenheim  and  Siemerling,  'Med.  Centralbl.,'  1889,  No.  39.]  It  is  inte- 
resting, however,  to  note  that  enlarged  muscular  fibres  have  been  found  in  the 
atrophied  muscles  in  infantile  pnralysis. 


600  SPINAL    CORD. 

it  is  found  after  death,  can  its  occurrence  be  regarded  as  beyond  ques- 
tion. ISTeTertheless  true  liypertropliy  of  fibres  is  a  change  tbat  may 
be  expected  to  be  occasionally  met  with  in  cases  that  are  due  to  a  con- 
genital tendency,  and  its  presence,  even  in  muscles  that  are  the  seat 
of  atrophy,  need,  therefore,  excite  no  surprise.  It  was  met  with  by 
Bruck  in  a  remarkable  case  of  general  muscular  enlargement  in  an 
idiotic  child,  in  whom  it  apparently  developed  after  birth.  The  affec- 
tion first  showed  itself  in  the  tongue,  but  the  muscles  presented 
spasmodic  contractions,  and  the  case,  like  most  of  the  kind,  was 
evidently  anomalous  in  its  features.  Muscular  hypertrophy  has  been 
found  in  the  singular  malady  described  in  the  next  section  (Thomsen's 
disease).  It  has  also  been  met  with,  in  very  rare  instances,  as  a 
wide-spread  or  partial  condition,  usually  associated,  strange  to  say,  with 
either  diminished  power  or  with  a  morbid  readiness  of  fatigue.*  The 
muscles  most  frequently  affected  have  been  those  of  the  shoulder  and 
upper  arm,  or  of  the  thigh  and  calf,  on  one  side  or  on  both.  It  has 
also  been  observed  in  the  glutei,  spinal  muscles,  and  trapezii.  The 
causes  are  obscure,  but  it  has  been  ascribed  to  over-exertion.  The 
diameter  of  the  fibres  has  been  increased  to  double  the  normal,  a 
maximum  of  -^-^  inch  having  been  met  with  (Eulenberg) ,  whereas  the 
normal  maximum  may  be  taken  as  -^^  inch.  An  increase  of  the 
nuclei  has  been  observed,  without  any  overgrowth  of  the  interstitial 
tissue. 

The  condition  is  manifested  by  an  increase  in  the  size  of  the  muscles, 
which  are  also  firm.  The  circumference  of  the  limb  is  greater  than 
normal,  and  when  the  change  is  unilateral  the  difference  between  the 
limbs  of  the  two  sides  may  be  very  striking.  The  maximum  circumfer- 
ence of  the  calf  has  been  as  much  as  seventeen  inches.  The  muscles 
are  soon  exhausted,  and  have  been  weak  in  some  cases,  while  in  others 
there  has  been  abnormal  strength  for  brief  exertion.  The  electrical, 
mechanical,  and  myotatic  irritability  of  the  muscles  has  usually  been 
found  unaltered. 

The  disease  may  be  suspected  if  a  marked  increase  in  size  in  an  adult 
is  accompanied  by  impaired  power  of  sustained  exertion,  if  the  muscles 
are  firm,  and  the  patient  does  not  manifest  other  indications  of  pseudo- 
hypertrophic paralysis.  The  diagnosis  can,  however,  only  be  made 
with  certainty  by  the  microscopical  examination  of  an  excised  fragment, 
and  even  then  subject  to  the  reservation  mentioned  on  the  previous 
page.  The  meagre  facts  regarding  the  course  of  the  disease  suggest 
that  it  usually  persists  without  getting  either  better  or  worse.  Treat- 
ment appears  to  have  but  little  influence  upon  it. 

A  smgular  case  has  been  reported  by  Eulenberg,t  which  differs  in 

*  Auerbach,  '  Virchow's  Archiv,*  Bd.  liii,  pp.  234  aud  397;  Berger,  'Deut.  Archiv 
f.  klin.  Med.,'  Bd.  ix,  1872,  p.  363  ;  Friedreich,  *l7eber  Prog.  Muskelatrophie,&c.,' 
1873;  Eulenberg,  '  Real-Eiicyclopadie,'  Bd.  ix,  p.  354.  A  very  aimilar  case  is 
described  by  Pal,  '  Wien.  klia.  Woehenschr.,'  1889,  No.  10. 

t  '  Deutsch.  med.  Woehenschrift,'  1885,  No.  12. 


thomsbn's  disease.  601 

many  particulars  from  the  form  of  disease  above  described,  and 
illustrates  the  complex  relations  of  enlargement  of  the  muscular  fibres 
and  its  connection  with  degenerative  processes.  A  man  aged  thirty- 
six  presented  an  enormous  enlargement  of  the  muscles  of  the  left 
leg,  which  were  soft  and  flabby  and  weak,  with  lowered  irritability. 
The  condition  had  slowly  developed  after  a  fall  on  the  back  at  ten, 
which  caused  imperfect  paraplegia,  motor  and  sensory.  A  year  later, 
during  pleurisy,  he  had  thrombosis  of  the  left  femoral  vein.  In  an 
excised  fragment  of  the  gastrocnemius  the  muscular  fibres  were  large, 
the  maximum  being  -^-^  of  an  inch ;  they  presented  fatty  and 
vitreous  degeneration,  and  fat  was  seen  between  the  fasciculi.  The 
muscles  of  the  other  leg  were  somewhat  wasted,  but  the  fibres  were  also 
enlarged  and  degenerated.  It  would  appear  as  though  the  condition 
had  resulted  from  a  traumatic  lesion  of  the  spinal  cord,  and  had  been 
intensified  in  the  left  leg  by  the  influence  of  the  thrombosis  and 
resulting  vascular  disturbance. 


THOMSBN'S  DISEASE;    MYOTONIA  CONGENITA. 

The  malady  thus  designated  may  be  considered  here,  notwith- 
standing the  obscurity  of  its  nature,  because  it  agrees  with  the 
diseases  last  described,  in  that  the  symptoms  are  muscular,  and  that 
the  disease  seems  often  congenital  and  occurs  in  families.  It  has 
been  named  after  the  physician  whose  description  gained  for  it  general 
notice,  and  who  is  himseK  its  subject,  but  it  had  been  previously 
described  by  Leyden,  and  hinted  at,  long  ago,  by  Sir  Charles  Bell.* 
It  is  not  always  congenital,  and  "  Transient  Myotone "  would  be  a 
more  exact  name. 

The  disease  is  characterised  by  a  peculiar  rigidity  of  the  muscles, 
which  comes  on  when  they  are  first  put  in  action  after  a  period  of  rest. 
The  rigidity  is  transient,  and  when  it  has  passed  off,  it  does  not  return 

*  Bell's  *  Nervous  System,'  Case  184,  p.  436.  The  objections  to  cognominal 
nomenclature  of  disease  are  certainly  i-educed  to  a  minimum  when  the  first  describer 
of  a  rare  disease  is  also  its  subject.  The  most  important  papers  on  the  disease  are 
those  of  Leyden,  '  Klinik  der  Ruckenmarkskr.,'  1874,  Bd,  i,  p.  128 ;  Thomsen,  'Arch. 
f.  Psyehiatrie,'  Bd.  vi,  1876,  p.  702,  also  '  Centralbl.  f .  Nervenkr.,'  1885,  p.  193 ; 
Bernhardt,  'Virchow's  Archiv,'  Bd.  Ixxv,  1879,  p.  516,  and  'Centralblatt  f. 
Nervenkr.,*  1885,  p.  122;  Ballet  and  Marie,  'Arch,  de  Neur.,'  1883,  No.  13; 
Mobius,  •  Schmidt's  Jahrb.,'  Bd.  cxcviii,  1883 ;  Ringer  and  Sainsbury,  '  Lancet,* 
18S4,  pp.  767,  816,  and  860 ;  Erb, '  Die  Thomsen'sche  Krankheit,'  Leipzig,  1886, 
also  *Deut.  Archiv  f.  klin.  Med.,'  1890,  xlv,  p.  529;  Self ert,  ib.,  xlvii;  Marie,  in 
'  Eucycl.  des  Sc.  Med.,'  1886 ;  Hale  White,  *  Brain,'  April,  1886,  and  '  Guy's  Hosp. 
Rt'p.,'  vol.  xlvi  (a  very  valuable  paper,  with  bibliography) ;  Jacoby,  Journ.  of 
Neur.  and  Ment.  Dis.,'  1887,  xiv,  p.  23  j  Jolly,  •  Siidwest.  Neurol^*  Baden,  June, 
1890;  Buzzard, '  Lancet,'  1887. 


G02  SPINAL  COED. 

as  long  as  exertion  is  continued.  Tlie  malady  is  often  hereditary,  and 
usually  affects  several  members  of  the  same  family.  In  that  of 
Thomsen,  cases  can  be  traced  through  five  generations.  It  appears 
to  be  more  common  in  Scandinavia  and  Germany  than  in  France 
or  England,  but  it  is  a  rare  disease ;  *  and  although  a  considerable 
number  of  clinical  cases  have  now  been  recorded,  there  has  been  no 
post-mortem  examination.  There  is  little  to  attract  attention  in  the 
slighter  forms,  and  the  rarity  of  these  may  Tiltimately  be  found  to  be 
less  than  at  present  appears. 

Both  sexes  suffer.  In  most  cases  the  symptoms  have  been  first 
noted  in  childhood,  between  four  and  ten  years  of  age,  sometimes 
even  in  the  cradle.  They  increase  during  the  period  of  muscular 
development,  and  then  remain  stationary.  A  few  patients  have 
seemed  free  until  about  the  period  of  puberty,  but  even  in  such  in- 
stances it  is  probable  that  the  tendency  was  congenital  in  origin, 
since  the  cases  occurred  in  families,  other  members  of  which  suffered 
earlier.  But  in  a  few  cases  symptoms  apparently  identical  with  those 
of  the  congenital  disease  have  come  on  in  early  manhood  after  some 
exciting  cause — in  one  case  a  lightning  stroke,  in  another  a  sudden, 
alarm. t  Severe  and  prolonged  exertion  during  two  years  preceded 
the  onset  of  very  characteristic  symptoms  in  a  man  of  twenty-five, 
without  heredity. J  In  such  cases  the  disease  has  been  apparently 
acquired. 

The  characteristic  symptom  is  tonic  spasm  of  the  muscles  when 
they  are  put  in  action  after  a  period  of  rest.  As  soon  as  the  patient 
attempts  to  move,  the  muscles  become  rigid.  The  rigidity  may  make 
movement  impossible  while  it  lasts,  or  may  merely  lessen  the  possible 
range  of  movement.  After  a  few  minutes,  or  less,  the  spasm  passes 
away,  to  be  renewed,  but  in  slighter  degree,  by  a  fresh  attempt.  If 
the  movements  are  continued,  the  spasm  soon  becomes  trifling,  and 
does  not  retiirn  until  after  a  period  of  rest.  The  longer  the  rest, 
the  more  troublesome  is  the  spasm.  The  subject  can  walk  for  hours 
without  fatigue  when  it  has  passed  off.  The  rigidity  is  sometimes 
lessened  by  alcohol  and  increased  by  attention  and  by  fear  of  it ;  the 
more  the  sufferer  tries  to  overcome  the  stiffness,  the  less  is  he  able  to 
do  so.  It  is  also  worse  in  cold  and  damp  weather.  In  a  severe  case,  a 
slight  impulse  will  make  the  person  fall,  and  it  may  then  be  impossible 
for  him  for  some  minutes  to  rise  from  the  ground.  The  arms  are 
usually  less  affected  than  the  legs,  but  in  some  cases  the  rigidity  fixes 
the  fingers  for  a  short  time  on  an  attempt  to  use  the  hand.  The 
muscles  of  the  face  are  usually  free ;  mastication  may,  however,  be 
iaterfered  with  by  the  spasm  in  the  muscles  of  the  jaw.      Earely,  the 

*  In  England  cases  have  been  observed  by  Buzzard,  Herschell,  Hale  White, 
Benham,  Chapman,  and  others. 

t  Engel, '  Phil.  Med.  Times,'  1883,  p.  412 ;  Scbonfeld,  *  Berlin,  med.  Wochenschr.,* 
1883,  No.  27. 

X  Moyer,  '  Med.  News,'  1890. 


thomsen's  disease.  603 

tongue  or  farv*  has  been  affected ;  still  more  rarely  the  muscles  of  the 
eyeball,  interf  *^ring  with  its  movement  and  retarding  the  descent  of  the 
upper  lid.*  Most  voluntary  muscles,  indeed,  seem  liable  to  suffer, 
even  the  musoles  of  the  larynx  and  pharynx  ;  those  concerned  in  the 
processes  of  respiration,  defaecation,  micturition,  and  coitus  have  also 
been  involv<jd.  Sometimes  the  spasm  is  greater  on  one  side  of  the 
body  than  01  the  other. 

The  muet'tes  are  always  well  nourished ;  they  are  often,  indeed,  above 
the  normal  size,  and  possess  more  than  normal  strength,  but  they 
are  sometimes  less  strong  than  is  normal,  even  when  large.  Thomsen 
believes  that  the  more  the  muscles  are  employed  the  less  severe  is 
the  spasm,  and  that  a  life  of  active  exertion  produces  some  per- 
manent amehoration  in  the  disease.  The  malady  is,  however,  a 
source  of  some  disability,  and  of  considerable  annoyance ;  in  the  words 
of  Thomsen,  "  it  casts  a  shadow  over  the  lives  of  the  sufferers,"  and 
may  have  caused  the  mental  irritability  and  hypochondriacal  tendency 
that  have  been  conspicuous  in  some  cases. 

Careful  investigations  of  the  muscular  phenomena  have  been  made 
by  several  observers,  and  that  by  Erb  is  especially  instructive.  In 
his  case  a  single  brief  effort  caused  tonic  contraction  which  lasted  for 
twenty-live  seconds.  Momentary  electrical  stimulation  of  the  nerves 
caused  only  a  momentary  contraction  of  the  muscles,  but  continued 
stimulation  always  causes  a  prolonged  contraction,  the  "myotonic 
reaction"  of  Erb.  In  certain  muscles,  moreover,  an  uninterrupted 
current  caused  peculiar  wave-like  contractions,  about  one  per  second, 
passing  from  the'  negative  to  the  positive  pole.f  Any  strong  stimu- 
lation of  the  muscle  itself  caused  a  prolonged  after-contraction.;}: 
The  irritability  of  the  nerves  is  generally  normal  in  degree ;  that  of 
the  muscles  is  normal  or  increased  to  voltaism.  In  some  cases  the 
anodal  closure  contraction  has  occurred  with  imdue  readiness,  and  the 
"latent  interval"  after  stimulation  has  been  found  unchanged  (Hale 
White)  or  increased. §  The  muscles  are  remarkably  sensitive  to 
mechanical  stimulation,  and  firm  pressure  may  cause  a  tonic  contrac- 
tion lasting  from  twelve  to  twenty  seconds. 

In  all  undoubted  cases  of  this  disease  sensibility  has  been  intact, 
and  the  superficial  reflexes  have  been  unaltered.  Myotatic  irritability 
is  normal  or  increased. 

Once  developed,  the  condition  seems  to  persist,  with  little  change, 
through  life,  but  in  one  case,  a  female  (in  whose  family  were  other 
cases),  considerable  improvement  is  said  to  have  followed  marriage. j| 

•  R;iyiuond,  •  Gaz.  Med.  de  Paris,'  June,  1891. 

f  .Although  Erb,  Seifert,  and  others  have  noted  this,  some  ohservers  (as  Hale 
White)  have  failed  to  obtain  the  phenomenon. 

I  Jolly  has  found  that  repeated  stimulations  by  either  current,  without  too  long 
ftn  interval  between  the  successive  stimulations,  tend  to  render  the  after-contraction 
less  and  less  evident,  until  it  ceases  to  occur  ('Nenr.  Centralbl.,'  1890,  p.  438). 

§  -025  or  -03  i^ec.  instead  of  -01  (Bluminau,  'Neur.  Cent.,'  1888,  p.  679). 

II  Herschell,  '  Lancet,'  Feb.  1, 1890. 


604  SPINAL   COED. 

In  some  instances  tlie  symptoms  are  slight,  and  remain  so,  even  wlien 
they  begin  in  early  life  and  occur  in  the  same  families  as  severe  cases. 
Such  cases,  when  isolated,  may  readily  be  overlooked. 

Pathologt. — An  examination  of  the  central  nervous  system  has 
yet  to  be  made,  but  it  is  unlikely  to  yield  results  of  novelty  or  im- 
portance. The  state  of  the  muscular  fibres  has  been  ascertained  from 
excised  fragments — a  method  which,  however,  involves  the  fallacy, 
already  mentioned  (p.  699,  note),  of  possibly  stimulating  the  fibres  to 
contraction.  At  the  same  time  the  evidence  of  hypertrophy  appears 
conclusive.  Where  the  minimum  width  was  the  same  as  in  health,  the 
maximum  diameter  was  double  the  normal  (Erb).  In  other  cases  the 
maximum  or  even  average  diameter  in  fragments  excised  under  chloro- 
form was  double  that  observed  after  death  in  una:ffected  persons ;  and 
that  the  size  was  pathological  was  rendered  probable  by  the  aspect 
of  the  fibres,  which  presented  indistinct  striation,  fewer  "  sarcous 
elements,"  irregular,  non-parallel  edges,  and  sometimes  vacuolation. 
An  increase  of  the  nuclei  and  of  the  interstitial  connective  tissue  has 
been  found.* 

The  manifestation  of  the  disease  is  in  a  disorder  of  the  functions  of 
the  muscles,  and  most  writers  on  the  subject  have  followed  Ley  den  in 
regarding  the  malady  as  essentially  muscular  in  nature,  as  consisting 
in  an  altered  functional  condition  of  the  muscular  tissue.  This 
opinion  is  strongly  supported  by  the  frequent  alteration  in  electrical 
excitability  (which  can  hardly  be  otherwise  interpreted  than  as  an 
indication  of  a  change  in  the  mode  of  action  of  the  contractile  muscular 
protoplasm),  by  the  structural  changes,  and  also  by  the  interesting 
experiments  of  Einger  and  Sainsbury,  who  found  that  certain  salts, 
such  as  sodium  phosphate,  are  capable  of  causing  in  the  frog  tonic 
spasm  bearing  considerable  resemblance  to  that  of  Thomson's  dis- 
ease, and  that  such  spasm  persists  not  only  after  the  nerve  has  been 
divided,  but  after  the  intra-muscular  nerve-endings  have  been  paralysed 
by  curara.  This  does  not  prove  that  Thomson's  disease  is  muscular 
in  orio-in ;  it  is  a  proof  that  a  similar  state  may  be  due  to  the  mus- 
cular tissue,  but  no  proof  that  this  is  the  sole  element  in  Thomson's 
disease.  We  must  remember  that  the  malady  has  apparently  resulted, 
in  rare  cases,  from  influences  acting  on  the  nervous  system  in  adult 
life,  and  also  that,  in  the  congenital  cases,  the  spasm  only  develops, 
as  a  rule,  after  some  years  of  normal  action.  It  may  thus  be  an 
acquired  disease,  and  the  case  related  at  the  end  of  this  chapter 
shows  that  similar  spasm  may  be  due  to  a  primary  affection  of  the 
cord.  The  spasm  is  a  transient  consequence  of  rest.  But  "rest"  is 
not  inactivity,  either  in  the  nerve-cells  or  muscles.  Muscular  "  tone" 
and  adaptation  to  posture  mean  an  unceasing  flow  of  nerve-force 
from  the  cells,  an  overflow  perhaps,  due  to  the  perpetual  elaboration 
of  energy  which  keeps  the  cells  ready  for  instant  response  to  the 

*  Erb,  Seifcrt,  and  also  Nearonow,  'St.  Petersburg  psych.  Gesellsch.,'  1889;  see 
•Neur.  Ceat.,'  1889,  p.  239.     Hale  White  found  no  increase  in  the  nuclei. 


thomsen's  disba.se.  605 

voluntary  stimTilus.  The  tonic  activity  then  ceases,  or  at  least  does 
not  interfere  with  the  different  action  excited  by  the  will.  But  the 
phenomena  of  Thomson's  disease  suggest  that  the  cells  respond 
abnormally  to  the  voluntary  stimulus,  that  this  causes  at  first  an 
increased  tonic  activity,  slowly  ceasing  as  their  energy  is  lessened  by 
action.  Wide  as  is  the  difference  between  the  muscular  and  nervous 
tissues,  we  must  remember  that  they  have  some  conditions  in  common. 
The  dependence  of  the  nutrition  of  the  muscles  on  that  of  the  motor 
nerve-fibres  and  cells  is  a  very  remarkable  fact,  and  so  also  is  the  in- 
fluence of  the  fxmctional  activity  of  the  cells  and  fibres  in  causing  a 
similar  condition  in  the  muscular  tissue.  To  say  that  the  two  struc- 
tures are  connected  is  hardly  an  explanation  of  the  fact.  Whatever  is 
the  nature  of  the  relation  between  them,  it  is  at  least  conceivable  that 
an  abnormal  functional  state,  congenital  in  origin,  may  be  common  to 
the  two,  and  that  the  peculiar  over-action  in  the  muscles  may  be 
accompanied  by  a  similar  over-action  in  the  ganglion-cells  of  the 
spinal  cord,  and  even  in  the  pyramidal  cells  of  the  cerebral  cortex. 
It  is  even  conceivable  that  the  condition  of  the  nerve-cells  may  be  the 
primary  change,  and  that  of  the  muscles  may  be  secondary,  although 
when  produced  it  is  in  some  degree  independent,  and  may  be  excited 
independently  by  local  stimulation.  Such  a  theory  enables  us  to 
•understand  the  two  facts  above  mentioned — the  influence  of  emotion 
and  the  acquisition  of  the  disease,  each  of  which  seems  to  be  incon- 
sistent with  a  purely  muscular  pathology. 

Treatment. — ISTo  treatment,  properly  so  called,  appears  to  exert 
any  influence  on  the  disease.  The  congenital  malady  persists  through 
life;  in  the  cases  in  which  a  similar  condition  has  apparently  been 
acquired,  it  has  also  been  persistent.  The  only  influence  that  has 
appeared  to  Thomsen  to  ameliorate  the  condition  is  a  life  of  active 
muscular  exertion.  But  it  is  possible  that  the  influence  of  thera- 
peutics is  not  yet  exhausted. 

Congenital  Paramyotone. — Under  this  name  Eulenberg  has  de- 
scribed *  a  strange  family  affection,  allied  to  Thomson's  disease  in  its 
general  character,  although  differing  very  much  in  its  special  features, 
and  equally  obscure  in  nature.  The  malady  was  widely  spread  in  the 
affected  family,  and  could  be  traced  through  six  generations,  but 
appears  now  to  be  dying  out.  Its  congenital  character  was  shown  not 
only  by  its  multiplicity,  but  also  by  the  fact  that  in  some  individuals 
it  was  manifested  immediately  after  birth.  The  symptom  was  tonic 
spasm,  lasting  from  a  quarter  of  an  hour  to  several  hours,  excited 
chiefly  by  cold,  although  often  by  merely  slight  cold.  The  rigidity  was 
followed  for  a  time  bj  weakness.  The  facial  muscles  were  very  prone 
to  become  thus  rigid,  especially  the  orbiculares  palpebrarum  and  oris; 
and  while  the  contraction  lasted  the  patient  was  often  unable  to  speak 
or  to  open  the  eyes.  The  rigidity  was  slighter  in  the  legs  than  in  the 
•  'Neurologisches  Centralblatt/  1886,  p.  265. 


606  SPINAL  COED, 

arms,  but  the  subsequent  weakness  was  equally  marked.  Warmth 
removed  the  spasm.  There  was  no  persistent  loss  of  power,  and  no 
increase  of  mechanical  irritability.  The  electrical  excitabihty  of  the 
nerves  was  normal;  that  of  the  muscles  was  lowered  to  each  current, 
and  there  was  an  abnormal  tendency  to  tetanic  contraction  during  the 
passage  of  the  current.  Eulenberg  speculates  that  the  symptoms 
may  be  due  to  reflex  vaso-niotor  spasm  in  the  muscles,  because  the 
diminution  of  the  blood- supply  to  muscles  renders  them  weak;  but 
it  is  clearly  equally  possible  that  the  two  certain  phenomena,  the 
sensory  impression  and  the  muscular  contraction,  may  be  directly 
connected. 

Ataxic  paramyotone  seems  the  best  provisional  designation  for  an 
acquired  condition  of  which  one  case  has  come  under  my  notice. 
Persistent  tonic  spasm,  like  the  transient  spasm  of  Thomsen's 
disease,  was  associated  with  distinct  ataxy,  and  also  with  weakness 
and  some  anaesthesia.  The  symptoms  commenced  gradually  in  the 
legs,  in  a  healthy  man  aged  40,  without  neurotic  heredity ;  it  invaded 
the  arms  six  months  later,  and  increased  more  rapidly  in  the  arms 
than  in  the  legs.  At  41^  his  state  was  this :  a  well-built  man,  with 
well-developed  muscles,  unduly  firm  in  all  parts  except  the  neck  and 
head.  The  firmness  was  due  to  tonic  spasm,  which  never  ceased.  It 
was  present  on  waking  in  the  morning,  and  through  the  day  interfered 
with  aU  movements,  making  them  slow  and  stiff.  He  rose  from  a  seat 
slowly  and  with  difficulty ;  when  standing  there  was  slight  unsteadi- 
ness, increased  by  closing  the  eyes,  and  a  slight  impulse  or  stumble 
in  walking  would  cause  a  fall,  chiefly  because  the  spasm  prevented 
the  needed  quickness  of  movement  to  save  him.  Prolonged  exertion 
had  no  influence  on  the  tonic  spasm,  which  opposed  passive  as  much 
as  active  movement.  Power  in  arms  and  legs  was  lessened,  but  not 
considerably.  The  grasp  =  50  and  55  ko.  The  extensors  of  the 
fingers  were  weaker  than  the  flexors,  but  could  act  perfectly  although 
involuntary  flexion  followed  the  slow  extension.  The  electrical 
irritabihty  of  the  muscles  seemed  to  be  normal.  Inco-ordination  was 
especially  marked  in  the  hands ;  with  eyes  shut,  buttoning  his  coat 
was  almost  impossible ;  the  attempt  was  made  with  thumb  and  fore- 
finger, the  others  being  flexed,  and  he  could  touch  his  nose  only  after 
many  wandering  failures.  On  the  palms,  from  the  wrist  to  the  tips 
of  the  fingers,  sensibihty  was  lost  to  touch,  slightly  delayed  to  pain, 
little  changed  to  temperature.  Slight  tactile  loss  extended  to  the 
back  of  the  last  phalanges;  on  the  back  of  the  hands  it  was  normal. 
A  similar  but  slighter  loss  existed  on  the  soles :  he  often  felt  as  if 
walking  on  a  rounded  surface.  The  size  and  weight  of  objects 
placed  in  the  extended  hand  could  not  be  recognised.  No  trace  of  the 
knee-jerk  or  other  evidence  of  myotatic  irritability  could  be  elicited, 
but  the  spasm  was  enough  to  explain  the  failure,  and  its  real  condition 
is  uncertain.    Mind,  special  senses,  and  cranial  nerves  were  unaffected. 


TUMOURS.  607 

Unfortunately  the  course  of  the  affection  could  not  be  observed. 
Its  symptoms  must  have  been  due  to  disease  of  the  spinal  cord,  and 
they  are  instructive  as  showing  that  muscular  spasm,  like  that  of 
Thomsen's  disease,  may  be  thus  produced.  The  time  may  not  have 
been  sufficient  for  the  production  of  changes  in  the  irritability  of  the 
muscles,  but  we  must  also  remember  that  differences  in  the  influence 
on  the  muscles  may  be  connected  with  the  defective  power. 


TUMOURS    OF   THE    SPINAL   CORD. 

Morbid  growths  within  the  spinal  canal  may  spring  from  the  mem- 
branes, or  may  grow  in  the  substance  of  the  cord  itself.  The  differ- 
ence in  seat  entails  some  difference  in  symptoms,  but  it  is  nevertheless 
convenient  to  consider  the  two  classes  together,  because  they  have' 
many  symptoms  in  common,  and  it  is  often  impossible  to  carry  the 
diagnosis  farther  than  the  existence  of  an  intra-spinal  tumour. 

Etiology. — The  general  causes  of  these  growths  correspond,  for  the 
most  part,  to  those  concerned  in  their  production  in  other  situations, 
and  present  few  peculiarities  that  merit  special  mention.  Fatty 
growth  outside  the  dura  mater  occurs  early  in  life ;  malignant  tumours 
develop  late.  Of  tumours  within  the  dural  sheath,  myxomata  are 
chiefly  met  with  in  middle  life,  tubercular  growths  occur  occasionally 
in  childhood,  but  generally  between  fifteen  and  thirty-five  (Herter  *)  ; 
lipomata  are  congenital.  Males  are  a  little  more  prone  to  suffer  than 
females,  t 

Of  the  diathetic  conditions  which  give  rise  to  tumours  elsewhere, 
only  two,  syphilis  and  tubercle,  are  effective  in  causing  growths  which 
commence  within  the  spinal  canal.  Parasitic  tumours  occur,  due  to 
the  same  influences  which  produce  them  in  other  situations.  A  few 
rare  growths  appear  to  be  congenital  in  origin,  due  to  the  abnormal 
development  of  germinal  tissue,  while  the  more  common  gliomata 
within  the  cord  arise  from  embryonal  tissue,  which  has  suffered  an 
arrest  of  its  normal  development.  Of  the  causes  of  other  forms  of 
tumour  we  know  practically  nothing.  Injuries,  such  as  a  blow  on  the 
spine,  have  been  supposed  to  be  occasional  causes,  and  their  influence 
in  rare  instances  has  seemed  possible,  but  the  evidence  is  not  so  strong 
as  it  is  in  the  case  of  tumours  in  some  other  situations.     In  many 

*  Heiter,  *  Journ.  of  Mentnl  Dis.,'  1890,  has  analysed  twenty-six  cases. 

t  A  collection  of  facts  relating  to  these  points,  by  Mr.  Victor  Horsley,  will  be 
found  in  the  paper  written  by  him  and  myself  on  the  case  of  successful  removal  of 
a  tumour  from  the  spinal  coid,  '  Med.-Chir.  Trans.,'  1889  j  but  the  numerical  basis 
ia  far  too  meagre  to  supply  more  than  statistical  suggestions.  It  will  be  necessary 
to  wait  for  a  considerable  time  before  adequate  data  are  obtained.  A  very  important 
paper  by  Allen  Starr  (*  Am.  Journ.  of  Med.  Sci.,'  1895,  Jane),  deals  with  an  addi. 
tional  sei  ies  of  cases. 


608 


SPINAL   CORD. 


cases  the  first  symptoms  have  immediately  followed  some  exposure  to 
cold  and  wet,  and  it  seems  probable  that  their  influence  may  have  excited 
secondary  processes  in  the  nerve-elements  which  were  already  deranged 
by  the  growth,  and  may  thus  have  frequently  evoked  the  first  sym- 
ptoms of  the  tumour,  but  it  can  have  had  no  share  in  the  production 
of  the  growth  itself.  Traumatic  haemorrhages  may,  however,  result 
in  cystic  formations,  which  have  been  mistaken  for  new  growths. 

Pathological  Anatomy. — The  growths  within  the  spinal  canal 
may  develop  outside  the  dura  mater,  inside  the  dura  mater,  or  within 
the  substance  of  the  cord.  The  extra-dural  tumours  may  spring  from 
the  membrane  or  from  the  tissue  between  the  membrane  and  the 
bone,  or  may  grow  into  the  canal  from  the  outside,  through  the 
intervertebral  foramina.  Subdural  tumours  may  proceed  from  the 
inner  surface  of  this  membrane,  from  the  arachnoid,  or  from  the  pia 
mater  (Fig.  176).  The  growths  in  the  cord  may  spring  from  the  pia 
.mater,  or  may  develop  in  the  substance  of  the  cord.  They  sometimes 
proceed  from  the  peculiar  tissue  which  surrounds  the  central  canal. 

The  forms  of  extra-dural  tumours  are  lipoma,  from  an  overgrowth 
of  the  fat  which  normally  exists  between  the  membranes  and  the  bone ; 
and  parasitic  tumours,  chiefly  echinococci ;  but  all  are  rare.*  Gl-rowths 
also  occur  that  spring  from  the  bones  or  intervertebral  tissue — enchon- 
droma,  sarcoma,  and  cancerous  tumours,  which  have  been  already 
considered.  Far  more  frequent  are  collections  of  inflammatory  pro- 
ducts from  bone  disease,  but  these  do  not  come  into  the  category  of 
morbid  growths. 


„^..^ 


Fia.  174. — Myolipoma  of  the  spinal  cord.  A,  transverse  section  of  the  tumour 
and  of  the  conus  meduUaris  of  the  spinal  cord,  to  which  the  growth  was 
attached,  and  of  which  the  grey  matter  is  represented  by  the  dotted  shading 
CM.  n,  sections  of  nerve-roots,  partly  enclosed  in  the  tumour ;  m,  bundles  of 
muscular  fibres.  B,  part  of  the  tumour  more  highly  magnified,  showing  the  fat- 
cells,  of  which  it  was  chiefly  composed;  jt.m.,  pia  mater  of  the  cord,  of  which 
I.e.  is  part  of  the  lateral  column.  C,  part  of  the  tumour,  still  more  magnified, 
showing  striated  muscular  fibres,  fibrous  tissue,  and  fat-cells  (see  also  Fig.  167, 
p.  580).     The  tumour  had  caused  no  symptoms. 


*  An  adeuo-sarcoma  has  also  been  met  with  (Hodenpyl,  '  Am.  Journ.  Med.  Sc..' 
1888). 


TUMOURS.  609 

The  tumoiirs  wMch  hegm  -witliin  the  dura-matral  sheath  are  chiefly 
syphilomata,  sarcomata,  and  myxomata,  sometimes  containing  cysts  or 
"  brain-sand."  Tubercular  and  parasitic  tumours  are  rare,  but  both 
echinococci  and  cysticerci  have  been  met  with,  developing  in  the 
meshes  of  the  arachnoid.  Fatty  tumours  have  been  foimd  in  a  few- 
instances,  and  may  contain  muscular  fibres.  They  are  congenital  and 
sometimes  associated  with  spina  bifida  (g.  v.).  Fig.  174  represents 
such  a  tumour,  a  myolipoma  consisting  of  fatty  tissue  and  striated 
muscular  fibres,  which  had  grown  from  the  pia  mater  or  meshes  of 
the  arachnoid,  and  was  found  in  a  case  of  tabes.*  Neuromata  occur 
on  the  nerve-roots,  and  may  compress  the  cord.f 

The  growths  within  the  substance  of  the  cord  itself  are  more 
diverse  in  character.  Syphiloma  and  glioma  are  the  most  common  ; 
sarcomata,  myxomata,  and  tubercular  tumours  also  occur,  containing 
the  characteristic  bacilli,  and  always  associated  with  tubercle  else- 
where. Hydi.tids  are  occasionally  met  with.J  Some  growths  have  a 
compound  character,  and  have  been  termed  myxo-sarcoma,  glio- 
sarcoma,  and  fibro-sarcoma.  Sarcomata  and  gliomata  are  sometimes 
very  vascular,  and  have  been  termed  "  angio-sarcoma  "  and  "  angio- 
glioma."  It  is  far  more  common  for  the  tumour  to  spring  from 
the  pia  mater  or  from  the  peri-ependymal  tissue  around  the  canal 
than  for  it  to  begin  among  the  nerve-structures,  but  tubercular 
growths,  usually  begin  in  the  substance  of  the  cord  and  usually  on  one 
side. 

Extra-dural  growths  are  always  single ;  those  within  the  dura  mater 
are  often  single,  but  sometimes  two  or  three  co-exist ;  or  the  growth 
may  be  diffuse,  as  in  a  sarcoma  of  the  pia  mater,  which  extended 
as  high  as  the  sixth  pair  of  cerebral  nerves,  and  as  low  as  the  cauda 
equina.  §  Neuromata  or  sarcomata  on  the  nerve-roots  are  often 
multiple,  and  sometimes  are  very  numerous.  Neuro-fibromata  on 
almost  all  the  nerves  of  the  body  have  been  observed  to  coincide  with 
a  similar  extra-dural  growth  compressing  the  spinal  cord.  Tumours 
within  the  spinal  cord  are  also  usxially  single,  even  those  of  tubercular 
nature,  but  occasionally  more  than  one  growth  is  found. 

The  size  attained  by  growths  outside  the  cord  is  necessaidly  moderate, 
in  consequence  of  the  limitation  of  the  space  in  which  they  grow. 
They  vary  from  the  size  of  a  pea  to  a  width  of  one  and  a  length 
of  two  inches,  which  is  rarely  exceeded,  and  chiefly  by  extra-dural 
tiunours.    Those  that  develop  from  the  pia  mater  are  usually  smaller 

*  See  'Path.  Soc.  Trans.,'  vol.  xxvii,  19.  Another  rare  case  is  one  of  lymph- 
angeioma  of  the  pia  mater  (Traube,  'Neur.  Cent.,'  1887). 

+  Myxo-fibromata  may  also  form  on  the  nerve-roots,  and  even  extend  on  to  the 
cord.     See  Bruce  and  Mott, '  Brain,'  July,  1887. 

X  Ransom  and  Anderson,  '  Brit.  Med.  Journ.,*  1892  (found  only  after  death 
though  operated  on),  and  a  case  under  my  care  in  which  the  hydatids  were  found 
and  removed  by  Mr.  Horsley  during  life.  In  other  cases  they  have  been  met  with 
post  mortem, 

§  Pasteur,  ♦  Brit.  Med.  Journ.,'  July,  1887. 

VOL.  I.  89 


610  SPINAL  CORD. 

tlian  those  that  spring  from  the  dura  mater.  Multiple  tumours  of 
the  nerve-roots  are  generally  small.  Adhesions  often  form  between 
the  growth  and  the  membrane  from  which  it  does  not  arise.  The 
tumour  compresses  the  nerve-roots  and  the  spinal  cord  (Figs.  176, 
178).  The  compression  produced  by  tumours  within  the  dui'a  mater 
is  always  greater  than  that  by  tumours  outside  this  membrane. 
The  amount  of  pressTire  is  proportioned  to  the  size  of  the  tumour  and 
its  consistence.  A  soft  growth  outside  the  sheath  may  attain  a  con- 
siderable size,  and  even  extend  through  the  intervertebral  foramina, 
without  exerting  much  pressure  on  the  cord  itself.  Even  such  exten- 
sion to  the  outside  seldom  causes  a  swelling  that  can  be  felt.  Probably 
the  Eontgen  rays  would  yield  useful  information.  At  the  compressed 
part  the  cord  is  narrowed  and  softened,  usually  indented  or  flattened, 
because  the  pressure  is  either  from  one  side,  from  the  front,  or  from 
the  back.  Rarely  the  degree  of  pressure  is  so  great  that  the  cord  is 
reduced  to  the  size  of  a  crow-quill,  and  it  has  even  been  apparently 
interrupted  at  the  spot,  the  upper  and  lower  portions  being  conical, 
and  their  pointed  extremities  connected  only  by  membranous  tissue. 
The  softening  of  the  cord  depends  on  inflammation,  the  "  pressure- 
myelitis  "  considered  in  a  preceding  chapter  (p.  390),  and  it  presents 
the  tissue  changes  that  have  been  there  described.  Secondary  iaflam- 
mation  may  extend  down  the  cord  below  the  growth,  or  for  a  short 
distance  above  it,  often  unequally  in  the  different  elements — sometimes 
ia  the  grey  matter  into  the  lumbar  enlargement,  and  even  on  one  side 
only  (Francotte,  see  p.  QV?,  footnote).  The  usual  secondary  degenera- 
tions are  commonly  conspicuous. 

The  growths  that  involve  the  cauda  equina  often  attain  a  larger  size 
than  those  that  occur  higher  up,  because  this  part  of  the  vertebral 
canal  is  large,  and  the  nerve-roots  occupy  but  a  small  part  of  it.  Most 
tumours  in  this  situation  are  sarcomata  or  fibro-sarcomata,  but  it  is 
a  not  uncommon  seat  of  syphilitic  growths.  They  usually  spring  from 
the  tissue  of  the  arachnoid,  and  often  surround  and  include  the  nerves 
(Fig.  179),  the  amount  of  damage  to  which  is  very  variable. 

Multiple  tumours  outside  the  cord  are  sometimes  very  numerous. 
They  are  usually  sarcomata,  and  spring  from  the  membranes  and 
sheaths  of  the  nerve-roots.  They  are  various  in  size,  ranging  from 
that  of  a  hazel-nut  to  that  of  a  pin's  head,  and  many  very  small  growths 
are  often  scattered  among  the  nerves  of  the  cauda  equina.  In  some 
cases  of  this  character  similar  growths  have  existed  in  the  cerebral 
membranes. 

The  tumours  within  the  spinal  cord  are  usually  small,  seldom  ex- 
ceeding half  an  inch  in  diameter,  and  usually  less,  even  when  they 
give  rise  to  considerable  impairment  of  function.  But  their  vertical 
extent  often  exceeds  their  transverse  diameter,  and  sometimes  they 
grow  through  a  considerable  extent  of  the  spinal  cord.  Gliomata,  and 
especially  the  gliomatous  growths  that  spring  from  the  central  region, 
and  arise  from  embryonal  neuroglial  tissue  (persistent  by  arrest  of 


Fio.  176. 


TUMODES. 
Fig.  176. 


611 


Pig.  177. 


fif'/N 


m 


Fig.  175. — Tumour  growing  from  the  inner  surface  of  the  dura  mater,  and  com- 
pressing tlie  spinal  cord  in  the  mid-dorsal  region.  The  tumour,  3  cm.  long,  was  a 
sarcoma  in  which  the  cells  were  arranged  in  concentric  groups,  the  centres  of  which 
Dad  undergone  calcification.  The  spinal  cord  was  softened  and  dark  in  tint  at  the 
compressed  psirt.*     (After  Lancereaux.) 

Fig.  176. — Sarcoma  growing  between  the  arachnoid  and  pia  mater  in  the  mid- 
dorsal  region  of  the  cord.  The  tumour  had  sprung  from  the  meshes  of  the  arach- 
noid, and  was  only  slightly  adherent  to  the  pia  mater.  The  spinal  cord  was 
compressed  and  softened.  In  the  figure  the  dura-matral  sheath  has  been  laid  opeu, 
except  at  tlie  top,  and  the  arachnoid  has  been  opened  over  the  lower  half  I'f  the 
tumour,  whi<h,  5  cm.  long,  lies  on  the  posterior  and  (right)  lateral  aspect  of  the 
cord.t     (After  Lancereaux.) 

Fig.  177. — A  tumour  (T)  growing  from  the  dura  mater,  and  compressing  the  riirht 
jide  of  the  spinal  cord  at  the  origin  of  the  4th,  5th,  and  6th  cervical  nerves.  Tlie 
tumour  was  a  spindle-celled  sarcoma  springing  from  the  arachnoid. J  (After  Lpyien.) 

*  The  patient  was  a  woman  seventy-one  years  of  age,  paraplegic,  with  stronc:  flexor 
contracture  of  the  legs,  loss  of  power  over  the  sphincters,  and  considerable  imjiair- 
meiit,  but  not  absolute  loss,  of  sensibility.  The  symptoms  came  on  gradually  six 
years  before  death.     (Lancereaux,  'Atlas  d'Anat.  Path.,'  p.  444.) 

t  The  jiatient  was  a  woman  twenty-eight  years  of  age.  The  first  symptom  was 
pain,  radiating  over  the  upper  part  of  the  abdomen  on  the  right  side,  and  aftfi- 
wards  extending  to  the  leg  and  to  the  left  side.  After  a  few  months,  wc:l<iic  s  of 
the  legs  gradually  came  on,  and  became  absolute,  with  loss  of  8eTisntioi\  anl  "1"  i"iu  cr 
over  the  sphincters.  Extensive  bed'-ores  were  the  immediate  cause  uf  (ieatli,  wiiich 
occurred  eighteen  months  after  the  on-et.     (Lancereaux,  'Atlas,'  p.  447.) 

X  The  patient  was  a  man  aged  thirty -five.     At  twenty-eight  pair,  comu.enced  iu 


612 


SPINAL    CORD. 


Fia,  178, 


Fig.  179. 


Fia.  ISO. 


Pig.  178. — Tumour  of  dura  mater  opposite  the  upper  part  of  the  lumbar  enlarge- 
ment, compressing  nerve-roots  and  spinal  (ord.  (From  a  preparation  in  University 
College  Museum.     Drawn  by  Dr.  H.  R.  Spencer.) 

Fig.  179. — Tumour  of  the  Cauda  equina  surionnding  and  enclosing  many  of  the 
nerve-roots.  The  cord  itself  was  unaffected.  The  structure  of  the  tumour  was 
that  of  a  fibro-sarcoma.* 

Fig.  180. — Neuromata  of  the  cauda  equina.  (After  Lancereaux.)  The  growths 
had  apparently  given  rise  to  no  symptoms. 

the  right  forearm,  and  continued,  intermitting,  for  some  years,  until  at  thirty -four 
it  extended  through  the  whole  arm  to  the  neck  beside  the  spine,  where  it  waa 
increased  by  movement.  Similar  pain  afterwards  came  on  in  the  left  arm.  Then 
followed  weakness  in  the  right  arm,  slight  spasm  in  the  right  leg,  and  tingling 
in  tiie  left.  The  arm  became  almost  powerless  and  wasted,  the  right  leg  weak, 
and  flexion  of  the  neck  caused  severe  local  pain.  These  symptoms  continued  and 
increased,  and  pain  in  the  left  leg  came  on.  The  cervical  spine  became  tender,  and 
movemeut  of  the  head  to  the  right  was  limited.  Then  both  legs  became  weak,  and 
sensibility  was  lost  as  high  as  the  nipples.  The  paraplegia  became  absolute,  bed- 
sores formed,  and  the  patient  died  seven  yeai-s  after  the  onset  of  the  symptoms. 
Softening  of  the  spinal  cord  extended  down  into  the  dorsal  region.  (Leyden,  *Klin. 
der  Riickenmarkskr.,'  Bd.  i,  p.  450.) 

*  Tlie  patient  was  a  man  aged  twenty-eight,  whose  symptoms  commenced  nine 
months  before  death,  and  consisted  in  severe  pains  in  the  legs  and  progressive  weak- 
ness, the  power  of  standing  being  lost  about  four  months  after  the  onset,  A  little 
power  in  the  flexors  of  the  liips  and  tlie  extensors  of  the  knees  persisted  almost  to 
the  last,  but  the  knee-jerk  was  lost.  All  the  muscles  of  the  legs  wasted,  those  below 
the  knee  extremely,  and  even  when  the  patient  was  first  seen  electrical  irritability 
was  greatly  lowered  to  both  currents,  without  any  reaction  of  degeneration.  Tactile 
sensibility  was  impaired  in  each  foot  and  lower  leg,  more  in  the  right  than  in  the 
left,  and  chiefly  in  the  region  supplied  from  the  sacral  plexus.  Sensibility  to  pain 
was  not  affected.  The  bladder  was  affected  early,  and  symptoms  of  p.velo-nephritis 
already  existed.  The  kidney  disease  was  the  immediate  cause  of  death,  which  was 
preceded  by  several  convulsions,  aiiparently  uraemic.  A  microscopical  examination 
showed  considerable  damage  to  many  nerve-roots  involved  in  the  tumour,  while 
others  had  escaped.  The  muscles  presented  intense  granular  degeneration  with 
some  longitudinal  striation,  and  increase  of  the  interstitial  nuclei. 


TUMOURS. 


fil3 


development,  and  often  associated  with  syringomyelia),  are  those 
which,  have  most  frequently  a  considerable  vertical  extent.  Such  a 
glioma  has  been  known  to  reach  from  the  medulla  oblongata  to  the 
lumbar  region,  and  often  extends  up  from  the  cervical  region  to  the 
base  of  the  brain. 

The  cord  is  enlarged  at  the  seat  of  a  tumour,  and  the  precise 
•character  of  the  enlargement  depends  on  the  position  of  the  growth. 
In  an  infiltrating  glioma  of  the  upper  part  of  the  cord  the  increase 
in  size  has  been  so  great  that  the  wall  of  the  foramen  magnum 
caused  a  constriction  around  the  swollen  cord.  Often  the  abnormal 
colour  of  the  tumour  is  perceptible  on  the  surface.  The  consist- 
ence of  the  cord  may  be  lessened  in  soft  growths,  but  the  tumour 
is  commonly  firmer  than  the 
normal  cord,  and  the  differ- 
ence is  frequently  increased 
by  softening  in  the  vicinity 
of  the  growth.  If  the  tumour 
reaches  the  surface  the  pia 
mater  and  arachnoid  may  be 
thickened  at  the  spot,  and 
they  may  even  be  adherent 
to  the  dura  mater,  but  there 
is  rarely  any  extension  of  the 
meningitis  to  other  parts. 
On  section  the  growth  is 
usually  veiy  distinct,  since 
its  aspect  contrasts  with 
that  of  the  nerve-substance. 
Only  glioma  and  myxoma 
resemble  the  normal  grey 
substance  in  appearance,  but 
the  new  tissue  is  usually 
distinct  in  position.  These 
tumours  in  some  cases  blend 
with  the  substance  of  the 
cord,  but  in  other  instances 
they  are  bounded  by  an  area 
of  softening  which  often  iso- 
lates even  invading  growths. 
Some  tumours  are  sharply 
limited.  It  may  be  difficult 
to  say  in  what  structure  the 
tumour  began,  but  this  may 
sometimes  be  ascertained  by 
an  examination  of  the  upper  and  lower  parts,  where  the  growth  is 
commonly  limited  to  that  part  of  the  cord  in  which  it  first  originated. 
Thus  in  Fig.  181,  B,  the  tumour  occupies  a  very  large  area,  although 


Fig.  181. — Tumour  of  spinal  cord,  springins 
iiom  the  tissue  around  the  centriil  canal. 
A,  upper  part  of  the  tumour  in  the  middle  of 
the  cervical  region  ;  the  growth  is  here  within 
the  posterior  commissure.  B,  section  through 
the  large.«t  part  of  the  tumour,  which  occupies 
the  whole  central  region  of  the  cord.  The 
vertical  extent  was  2  cm.  It  was  a  sarcoma 
composed  chiefly  of  spindle-cells  arranged 
in  fasciculi  and  in  concentric  layers.  The 
patient  was  suffering  also  from  a  cerebral 
tumour  causing  hemiplegia,  and  tlie  spinal 
growth,  which  apparently  developed  rapidly 
during  the  final  period  of  pals\  and  prostra- 
tion, was  not  suspected  during  life. 


614 


SPINAL    CORD. 


the  nearly  equal  extent  on  eacli  side  suggests  its  central  origin,  and 
this  is  clearly  seen  in  the  section  through  the  upper  part  of  the  growth, 
in  which  the  posterior  commissure  can  be  traced  in  front  and 
behind  the  small  area  of  growth,  pointing  to  the  peri-ependy- 
mal  tissue  as  its  source.  Syphilitic  tumours  commonly  originate 
from  the  pia  mater,  as  they  do  also  in  the  brain,  and  the  mem- 
brane in  their  vicinity  is  often  thickened.  They  both  invade  and 
compress  the  nerve-tissue.  An  instance  of  such  a  gumma  of  the  cord 
is  shown  in  Fig.  182.     It  probably  commenced  at  the  furrow  opposite 


Fig.  182. — Syphiloma  in  the  Bpinal  cord,  occupvirg  the  position  of  the 
posterior  cornu  and  adjacent  parts  of  the  posterior  and  lateral  columns,  in 
the  lower  cervical  region.  Tlie  tumour  presented  the  usual  comhination 
of  grey  translucent,  and  yellow  cheesy  areas.  A  second  similar,  hut  much 
smaller  tumour  existed  higher  np.  Tiie  symptoms  were  complicated  by 
hemiplegia,  &c.,  of  cerebral  origin,  due  to  a  gumma  in  the  brain.  Para- 
lysis with  rigid  flexor  contracture  of  the  left  arm  and  leg  were  apparently 
due  to  the  growth.  The  disease  was  certainly  syphilitic,  but  it  is  note- 
worthy that  it  had  developed  during  continuous  treatment  by  full  doseg 
of  iodide,  which  had  entirely  removed  earlier  symptoms,  but  to  which  the 
system  had  apparently  become  accustomed. 


the  head  of  the  posterior  cornu,  which  it  has  destroyed,  and  the  pres- 
sure which  it  exerted  is  evident  from  the  displacement  of  the  posterior 
median  septum.  In  aE  foirms  of  tumour  the  central  canal  is  often 
obliterated  by  the  pressure,  and  may  be  slightly  dilated  higher  up; 
this  dilatation  is  especially  common  from  growths  that  begin  in  the 
peri-ependymal  tissue.  The  considerable  enlargement  of  the  canal, 
termed  "  syringomyelia,"  is  generally  associated  with  a  peculiar 
growth  in  this  position,  probably  congenital  in  origin ;  the  condition 
is  described  in  another  chapter.  In  other  forms  of  tumour,  cysts 
occasionally  develop,  and  haemorrhage  may  occur  into  such  cysts,  or 
into  the  softened  tissue  in  the  vicinity  of  the  tumour,  or  even  in  the 


TUMOURS.  615 

substance  of  a  soft  growtli  itself,  especially  when  this  is  a  glioma. 
From  the  region  of  the  growth,  secondary  degenerations  can  frequently 
be  traced  upwards  and  downwards,  but  these  are  often  slighter  than 
the  apparent  damage  would  suggest,  sometimes  by  reason  of  the 
remarkable  tolerance  of  the  nerve-fibres  to  pressure  if  this  is  slowly 
developed,  sometimes  because  a  rapidly  grooving  tumour  has  not  given 
time  for  them  to  become  conspicuous.  As  regards  vertical  position, 
growths  mny  occur  in  any  part,  but  are  said  to  be  most  frequent 
beneath  the  lower  cervical  spines,  and  in  the  upper  and  lower  dorsal 
regions,  because  (it  is  conjectured)  there  is  least  room  at  these 
places,  although  why  the  result  should  follow  from  the  cause  is  not 
clear. 

Symptoms. — The  symptoms  produced  by  tumours  within  the  spinal 
canal,  whether  these  are  within  the  cord  or  outside  it,  resemble  in 
their  general  characters  those  which  we  have  already  considered  as 
resulting  from  growth  and  caries  ui  the  bones  of  the  vertebral  column. 
The  resemblance  is  most  close  in  the  case  of  extra-dural  growths,  and 
least  so  in  that  of  tumours  within  the  substance  of  the  cord.  The 
chief  differences  are  the  relative  preponderance  of  symptoms  of  early 
irritation  of  the  cord  itself  when  the  growth  is  within  it,  and  the 
frequent  indications  of  a  transverse  extension  of  the  damage  from  one 
side  of  the  cord  to  the  other. 

In  the  majority  of  cases  pain  is  prominent  through  the  whole  course 
of  the  disease.  It  is  usually  the  earliest  effect,  severe  both  before  and 
after  the  development  of  other  symptoms.  The  pain  may  be  intense 
along  the  course  of  the  nerves  which  arise  at  the  level  of  the  tumour, 
and  also  in  the  parts ,  supplied  by  nerves  below  that  level,  e.  g.  in  the 
legs,  but  it  is  not  felt  above  the  growth.  It  may  be  a  sharp  acute 
pain,  but  has  often  a  "burning"  character,  and  is  sometimes  "  stabbing" 
or  "rending."  Dull  aching  pain  may  be  felt  between  the  attacks  of 
severe  pain,  or  before  them,  and  may  then  give  rise  to  the  most 
frequent  of  all  diagnostic  errors — mistaking  grave  organic  disease  for 
simple  rheumatism,  on  account  of  the  similarity  in  the  character  of  the 
pains.  The  intensity  of  the  severe  pain  is  usually  very  great,  and  it 
has  more  than  once  led  the  unhappy  sufferer  to  attempt  suicide.  The 
pains  are  often  felt  on  one  side  first,  in  one  arm,  in  one  side  of  the 
trunk  at  a  given  level,  or  in  one  leg,  and  a  considerable  time  may 
elapse  before  they  extend  to  the  other  side.  Occasionally  the  pain 
is  bilateral  from  the  first.  Dr.  Starr  has  drawn  attention  to  the 
rarity  of  tenderness  of  the  nerves  in  the  line  of  the  pain.*  It  is 
generally  greatest  when  the  tumour  compresses  the  cord  from  behind 
forwards,  or  vice  versa  (Horsley)  ;  this  is  open  to  more  than  one 
explanation.  Pain  is  sometimes  increased  by  movement,  but  rarely 
in  the  intense  degree  common  in  tumours  of  the  bone.  When  the 
growth  is  in  the  lower  lumbar  region,  the  pain  is  often  first  referred 
to  the  soles,  and  seems  to  ascend.  The  sensory  loss,  due  to  subse- 
•  Allen  Starr,  « Amer.  Journ.  Med.  Sc.,'  1895. 


616  SPINAL    CORD. 

quent  nerve  destruction,  follows  a  similar  course.  Pain  is  sometimes 
felt  in  the  spine,  frequently  wlien  tlie  growth  begins  in  the  dura 
mater,  rarely  when  it  is  within  the  substance  of  the  cord.  Tenderness 
of  the  spine  often  exists  in  the  former  cases,  but  is  on  the  whole 
not  common.  Other  subjective  sensations  may  occur  with  the  pain 
or  in  the  intervals — "  numbness,"  tingling,  formication,  &c.,  and 
they  give  significance  to  the  pains  as  an  indication  of  organic  damage 
to  the  nerve- structures.  The  root-pains  in  the  trunk  are  often  accom- 
panied by  a  sense  of  constriction,  which  may  be  very  distressing. 
Hyperaesthesia  of  the  skin  frequently  accompanies  pain  felt  at  the 
level  of  the  tumour,  less  commonly  that  in  the  parts  below.  Yerj 
rarely  pain  is  absent;  chiefly  in  extra-dural  lipoma,  probably  from 
the  nature  of  the  growth. 

Muscular  spasm  is  another  common  symptom,  most  pronounced 
"when  the  tumour  springs  from  the  membranes.  There  may  be  some 
rigidity  of  the  back  opposite  the  seat  of  the  growth,  usually  associated 
with  local  pain.  It  is  most  marked  when  the  disease  is  at  the  more 
mobile  parts  of  the  spine,  especially  when  it  is  in  the  cervical  region. 
In  such  cases  the  rigidity  may  be  painful,  and  the  pain  may  be 
increased  by  movement.  Severe  spasm  in  the  abdominal  muscles  is 
often  associated  with  severe  girdle-pain.  Contractures  may  develop 
in  the  limbs,  both  in  those  supplied  by  the  nerves  which  arise  at  the 
level  of  the  tumour,  and  are  directly  irritated  by  it,  and  also,  although 
less  commonly,  in  the  parts  supplied  from  the  spinal  cord  below  the 
level  of  the  growth.  Thus  the  tumour  in  one  half  of  the  cord  in  the 
cervical  region  (shown  in  Fig.  182)  caused  persistent  flexor  contracture 
in  the  arm  and  leg  on  the  side  of  the  growth, -and  in  the  early  stage 
of  dorsal  tumours  one  leg  only  may  be  rigid,  or  more  so  than  the 
other.  The  seat  of  rigidity  thus  needs  to  be  specially  noted,  as  well 
as  its  character,  if  we  are  to  obtain  from  it  diagnostic  guidance, 
because  it  may  be  due  to  the  irritation  either  of  the  nerve-roots  or 
of  the  conducting  tracts.  Both  forms  sometimes  occur  before  loss  of 
power,  but  pain  generally  precedes  them. 

Paralysis,  of  gradual  onset,  is  almost  invariably  one  of  the  sym- 
ptoms. Paraplegia  is  the  most  common  form,  but  all  four  limbs  may 
be  paralysed  by  a  growth  in  the  cervical  region.  Yery  commonly  one 
leg  becomes  weakened  before  the  other,  and  occasionally  one  arm  and 
leg  suffer  before  those  of  the  other  side.  Tumours  which  are  situated 
in  the  middle  line  or  in  the  centre  of  the  cord,  however,  usually 
affect  both  sides  at  the  same  time,  and  the  effects  of  a  congenital 
growth  may  thus  accompany  and  complicate  those  of  an  associated 
syringomyelia  (g.  v.).  Ihe  proximal  parts  of  the  limbs  seem,  as  a 
rule,  to  be  first  affected,  although  exceptions  are  occasionally  met 
with. 

The  palsy,  gradual  in  onset,  is  usually  also  slow  in  its  extension,  the 
rate  of  its  increase  depending  on  the  rate  at  which  the  tumour  grows 
and  compresses  the  spinal  cord.     This  is  the  characteristic  course  of 


TUMOUES.  617 

tlie  paralysis  produced  by  a  tumotir.  But  in  a  large  numlDer  of  cases 
the  loss  of  power  comes  on  in  a  subacute,  or  even  an  acute  manner,  in 
consequence  of  the  myelitis  excited  by  the  compression,  which  in  such 
■cases  may  run  a  course  of  independent  rapidity.  When  the  inflam- 
mation is  thus  in  excess  of  its  cause,  the  j^alsy  may  lessen  as  damage 
from  the  inflammation  subsides  to  the  degree  of  the  damage  from 
pressure.  The  symptoms  that  depend  on  compression  slowly  but 
surely  increase  as  the  tumour  grows,  and  usually  attain  a  high  degree 
of  intensity ;  the  loss  of  power  in  the  legs,  for  instance,  becoming  abso- 
lute and  remaining  so. 

Loss  of  sensation  may  come  on  with  the  loss  of  motor  power; 
more  often  after  this  has  become  considerable.  It  may  be  slight  or 
partial  at  first,  occasionally  for  a  long  time,  but  tends  to  become 
complete  as  the  damage  to  the  cord  increases,  and  it  often  ascends 
the  legs.  It  corresponds  in  distribution  to  the  motor  palsy  when  the 
disease  is  below  the  middle  of  the  dorsal  region ;  but  if  situated 
higher,  and  on  one  side,  the  sensory  loss  is  often  greater  on  the  side 
opposite  to  the  motor  paralysis.  Tumours  of  the  cord  furnish  a  con- 
siderable proportion  of  the  cases  in  which  the  crossed  symptoms  of  a 
unilateral  lesion  are  well  marked  (see  p.  272).  In  addition  to  the  loss 
of  sensation  which  results  from  the  damage  to  the  cord  itself,  areas  of 
anaesthesia  may  exist  in  the  region  supplied  by  the  nerves  which  are 
damaged  by  the  growth,  and  in  which  the  severe  radiating  pains  are 
felt.  If  there  is  crossed  sensory  and  motor  palsy,  these  root  symptoms 
exist,  chiefly  on  the  side  opposite  to  the  anaesthesia  in  the  limbs. 
Inco-ordination  of  movement  is  not  a  common  symptom  of  spinal 
tumours,  but  has  been  occasionally  met  with  in  cases  of  growth 
invading  the  posterior  columns,  and  also  as  a  result  of  multiple 
tumoui's  of  the  nerve-roots  (see  p.  478).  Loss  of  power  usually 
co-exists  and  quickly  becomes  predominant  when  the  growth  is  in  the 
cord. 

The  condition  of  refl.ex  action  depends  on  the  position  of  the 
tumour.  In  the  lumbar  enlargement  or  the  cauda  equina  a  growl  h 
abolishes  reflex  action  in  the  legs,  and  in.  any  position  the  trunk- 
reflexes  are  lost  at  the  level  of  the  lesion.  But  when  the  disease  is 
situated  higher  up,  reflex  action  is  increased,  and  the  excess  of 
cutaneous  reflex  action  is  a  very  marked  and  early  symj)tom  of  the 
disease ;  it  is  lessened  and  lost  only  when  the  lower  part  of  the  cord  is 
damaged  Tjy  secondary  myelitis  (superadded  to  descending  degenera- 
tion and  distinct  from  it).  The  myotatic  irritability  presents  also  the 
increase  that  invariably  results  from  damage  to  the  pyramidal  tractsj 
and  the  usual  tonic  spasm  also  gradually  develops  and  often  reaches  a 
high  degree.  Its  loss,  with  muscular  relaxation,  sometimes  met  with 
under  these  conditions,  depends  on  a  descending  myelitis,  sometimes 
slight    and    partial.*      Muscular    contracture    is    superadded    more 

*  As  was  found  to  be  -the  case  by  Francotte  ('Fibrome  de  la  Dnre-mere  spiimle,' 
JjihgBf  1888)  in  a  case  of  tumour  compressing  the  upper  dorsal  region.     Sliglit 


618  SPINAL   CORD. 

frequently  in  ttiinotirs  than  in  many  other  diseases,  and  rigid  spasm 
in  the  flexors  of  the  knee  and  hip  may  keep  the  heels  in  contact  "with 
the  nates. 

Wasting  of  the  muscles  is  for  the  most  part  confined  to  the  regions 
supplied  by  the  damaged  nerve-roots.  In  tumours  of  the  lumbar 
enlargement  and  cauda  equina  the  atrophy  of  the  muscles  of  the  legs 
is  a  very  conspicuous  symptom.  Considerable  muscular  wasting  in 
the  legs  may  result  from  an  irritable  growth  in  the  cord  above  the 
lumbar  enlargement,  even  in  the  cervical  region,  (but  such  atrophy 
does  not  reach  the  extreme  degree  produced  by  growths  in  the  lumbar 
enlargement  or  cauda  equina,)  and  this  condition  is  further  dis- 
tinguished by  the  preservation  of  the  muscle-reflex  actions  (knee- 
jerk,  &c.),  and  often  by  their  excess  (foot- clonus).  Yaso-motor  dis- 
turbance sometimes  occurs  in  the  early  stage  of  the  disease,  chiefly 
in  the  distribution  of  irritated  roots,  where  flushing  of  the  skin  has 
been  noted,  and  the  so-called  "meningitic  streak"  ("tache  spindle") 
may  often  be  produced.  Occasionally  vaso-motor  oedema  is  a  con- 
spicuous early  symptom.  In  the  later  stages  bedsores  are  very 
common  and  often  severe.  Dilatation  or  contraction  of  the  pupil  is 
common  when  a  tumour  is  situated  in  the  lower  cervical  region.  Loss 
of  control  over  the  sphincters  usually  accompanies  motor  palsy  in  the 
legs;  their  state  corresponds  with  that  of  reflex  action  (p.  267). 

The  course  of  the  symptoms  is  variable,  and  depends  on  the  rapidity 
of  growth  of  the  tumour,  and  on  the  share  which  secondary  myelitis 
takes  in  the  production  of  the  symptoms.  As  a  rule  the  root  symptoms 
occur  first,  and  in  growths  outside  the  cord  they  may  exist  alone  for 
months,  and  even,  in  the  case  of  a  slowly  growing  tumour,  for  yeara 
before  the  symptoms  of  damage  to  the  cord  itself  are  added.  On  the 
other  hand,  they  may  be  entirely  absent  in  growths  which  commence 
within  the  cord  itself.  In  some  cases  the  symptoms  are  steadily 
progressive  from  first  to  last.  In  others  fie  progress  is  intermittent  j 
periods  of  increase  in  the  symptoms  alternate  with  stationary  periods. 
Their  course  is  variable,  even  in  cases  in  which  the  seat  of  the  tumour 
and  its  nature  are  the  same,  because  they  depend  on  the  secondary 
changes  in  the  nerve-elements,  and  the  course  of  these  (such  as  the 
myelitis  induced  by  pressure)  is  subject  to  variations,  the  cause  of 
which  is  often  indistinct.  Thus  the  chronic  course  of  the  symptoms 
is  often  varied  by  periods  of  rapid  and  even  sudden  increase,  espe- 
cially in  the  case  of  growths  that  involve  the  spinal  cord  itself ;  and 
symptoms  that  have  thus  developed  with  rapidity  are  apt  (as  already 
mentioned)  to  subside,  if  not  completely,  at  least  in  considerable 
degree.  Hence  a  rapid  increase  of  the  cord  symptoms  may  be 
followed  by  positive  improvement  for  a  time,  but  scarcely  ever  by  an 

descending  inflammation  of  the  right  anterior  cornu  coincided  with  flaccid  palsy  of 
the  right  leg.  It  should  have  heen  mentioned  that  the  connection  of  a  similar  loss  of 
myotatic  irritability  with  descending  inflammation  in  dorsal  myelitis  (referred  to  at 
pp.264,  271  and  360)  has  been  proved  in  two  other  cases  by  Francotte  (loc.  cit.,  p.  9).. 


TUMOURS.  619 

actual  disappearance  of  the  manifestations  of  the  disease.  The  most 
important  element  in  the  com-se  of  the  disease  is  the  tendency  to  a 
lateral  extension  of  the  symptoms,  the  expression  of  the  transverse 
extension  of  the  damage,  as  the  several  structures  of  the  cord,  at  the 
same  level,  successively  suffer.  Although  there  is  sometimes  an  exten- 
sion dovniwards  of  the  interference  v^ith  the  central  functions  of  the 
cord,  when  secondary  myelitis  descends  to  the  lumbar  enlargement, 
there  is  scarcely  ever  an  extension  upwards  above  the  level  of  the 
initial  interference  with  function.  A  very  small,  slowly  growing  tumour, 
even  vsdthin  the  cord,  sometimes  causes  no  symptoms. 

The  chief  differences  between  the  symptoms  of  tumours  of  the  cord 
and  membranes,  besides  those  mentioned  on  p.  615,  are  that,  in 
the  latter,  the  root  symptoms  are  more  often  severe,  and  that  the  cord 
symptoms  occur  later,  and  are  at  fii-st  more  limited  in  extent.  Spinal 
pain,  tenderness,  and  rigidity  are  more  common.  In  cord  tumours  the 
radiating  pains,  due  to  irritation  of  the  nerve-roots,  are  more  often 
trifling  or  absent,  unless  the  growth  begins  in  the  neighbourhood  of 
the  posterior  horn ;  then  they  may  be  pronounced.  The  cord  symptoms 
are  often  bilateral  from  the  first,  and  in  central  tumours  they  may 
develop  equally  on  the  two  sides.  If  they  are  unilateral  at  the  onset 
they  extend  to  the  other  side  sooner  than  in  the  case  of  meningeal 
growths.  In  the  case  of  tubercular  growths  the  symptoms  of  irrita- 
tion are  usually  subordinate  to  those  of  impaired  conduction,  and 
pain  is  felt  chiefly  in  the  legs  (Herter).  Extensive  muscular  atrophy 
is  more  common  in  tumours  of  the  cord  than  in  those  of  the  membranes 
(except  in  the  case  of  tumours  of  the  cauda  equina),  because  central 
tumours  may  damage  the  grey  matter  through  a  considerable  vertical 
extent,  and  are  most  common  in  the  enlargements,  and  also  because 
myelitis  spreads  in  the  grey  matter  far  more  frequently  when  the 
exciting  growth  is  in  the  cord  itself,  than  when  it  is  in  the  mem- 
branes. 

In  tumours  of  the  cervical  region,  the  interference  with  movement 
of  the  neck,  from  the  rigidity  of  the  muscles,  is  often  well  marked, 
especially  when  the  tumour  is  in  the  membranes.  The  pains  due  to 
irritation  of  the  nerves  are  felt  in  the  arms,  and  are  often  associated 
with  muscular  atrophy.  The  excess  of  reflex  action  in  the  legs  is  very 
great,  and  if  the  influence  of  the  tumour  is  chiefly  on  one  side, 
hemiplegic  weakness,  with  crossed  anaesthesia,  is  present. 

Growths  in  the  dorsal  region  of  the  cord  usually  cause  severe 
radiating  pains  in  the  trunk,  often  accompanied  by  an  intensely  painful 
sense  of  constriction,  by  hyperaesthesia,  and  sometimes  by  anaesthesia. 
The  simple,  merely  sloping  course  of  the  dorsal  nerves  reveals  the  pre- 
cise position  of  the  disease  more  clearly  than  do  the  symptoms  in  the 
arms  when  the  tumour  is  in  the  cervical  region.  The  interference  with 
the  trunk-reflexes  often  corroborates  the  indications  afforded  by  the 
subjective  symptoms.  Spinal  tenderness  may  be  also  present,  but 
rigidity  of  the  spinal  muscles  is  not  common.    Eeflex  action  in  the 


620  SPINAL  COED. 

legs  presents  the  same  excess  tliat  results  frora  turaours  in  the  cervical 
region,  but  sometimes,  especially  when  the  growth  is  near  the  lumbar 
enlargement,  or  the  pressure  myelitis  has  been  acute,  the  descending 
myehtis,  as  we  have  seen,  may  abolish  the  reflex  action  and  remove 
spasm,  if  this  previously  existed. 

The  symptoms  produced  by  tumours  which  involve  the  lumbar 
enlargement  vary  considerably  according  to  the  precise  position  of 
the  growth.  The  characteristic  pains  are  felt  in  the  legs,  and  the 
muscles  suppHed  from  the  part  invaded  or  compressed  undergo 
atrophy,  and  present  loss  of  reflex  action  and  of  myotatic  irritabihty ; 
but  the  anassthesia  is  often  Hmited  in  area.  A  growth  in  the  upper 
part  of  the  enlargement  may  cause  wasting  only  in  the  flexors  of  the 
hip  and  extensors  of  the  hnee,  with  loss  of  the  knee-jerk  and  anaes- 
thesia in  the  front  of  the  thigh,  while  the  muscles  below  the  knee 
present  simple  palsy  with  myotatic  excess.  Thus  a  man,  who  had  had 
syphilis,  complained  of  pain  in  the  left  groin  and  thigh,  and  presented 
wasting  of  the  extensors  of  the  knee,  with  anaesthesia  in  the  front 
of  the  thigh ;  there  was  loss  of  the  left  knee-jerk,  and  a  foot-clonus 
on  each  side.  He  recovered  partially,  but  there  was  doubtless  a 
small  syphiloma  at  the  upper  part  of  the  lumbar  enlargement  on 
the  left  side,  interrupting  the  reflex  arc,  damaging  the  left  motor  and 
sensory  roots,  and  compressing  both  pyramidal  tracts.  A  growth  in 
the  membranes  adjacent  to  the  middle  or  lower  part  of  the  lumbar 
enlargement  usually  causes  symptoms  of  wide  distribution,  affecting 
the  whole  of  both  legs,  and  the  early  symptoms  of  irritation  often 
involve  the  distribution  of  the  upper  lumbar  nerves.  But  a  growth 
at  the  same  level  within  the  cord  may  affect  chiefly  the  muscles  of  the 
lower  leg,  and  cause  anaesthesia  in  the  region  supplied  from  the  sacral 
plexus.  There  may  also  be  palsy  and  wasting  of  all  the  muscles  of 
both  legs,  rom  extensive  secondary  myelitis,  but  even  then  the  sensory 
loss  is  usually  limited.  In  lumbar  tumours  there  is  early  palsy  of  the 
bladder  and  sphincters.  Examination  of  the  sphincter  ani  shows 
absolute  paralysis,  and  not  the  pure  reflex  action  present  when  the 
disease,  arresting  voluntary  influences,  is  higher  up  the  cord.  The 
tendency  to  bedsores  is  commonly  strong  in  tumours  of  this  part. 

Growths  situated  in  the  cauda  equina,  which  are  below,  and  do  not 
affect,  the  cord  itself,  produce  symptoms  very  similar  to  those  due  to 
tumours  of  the  lumbar  enlargement,  but  the  symptoms  are  bilateral 
from  the  first  in  the  majority  of  cases.  Earely  one  leg  is  affected 
alone  at  the  outset.  The  symptoms  usually  begin  in  the  lowest  parts 
of  the  legs,  which  are  throu^-hout, involved  in  greater  degree  than  the 
upper  parts,  and  the  flexors  of  the  hip  may  escape  altogether. 
Muscular  contraction  is  much  less  common  than  in  tumours  situated 
higher  up.  The  anaesthesia  is  chiefly  in  the  region  supplied  by  the 
sacral  plexus ;  the  pains,  usually  very  severe,  are  first  felt  in  the  same 
region,  and  there  may  be  severe  pain  on  each  side  of  the  sacrum 
radiating  to  the  region  of  the  sciatic  nerves.     The  muscles  below  the 


TUMODRS.  G21 

knee  rapidly  waste,  and  often  (but  not  always)  present  tlie  degenera- 
tive reaction.  These  symptoms  were  well  marked  and  cliaracteristic 
in  the  case  figured  and  described  at  p.  612  (Fig.  179),  and  tlie  condi- 
tion of  the  muscles  suggests  that  the  absence  of  any  voltaic  irritability 
in  the  muscular  fibres  was  -due  to  their  early  and  intense  fatty  degene- 
ration. In  many  cases  the  symptoms  are  irregular  in  distribution,  the 
nerves  are  included  in  the  growth,  and  are  often  damaged  unequally, 
sometimes  less  than  might  be  expected  from  the  size  of  the  tumour, 
which  may  be  considerable  before  its  expansion  is  arrested  by  contact 
with  the  bony  walls  of  the  canal.  Hence  the  loss  of  sensation  may  be 
partial,  and  reflex  action  may  not  be  completely  abolished.  In  the 
case  above  mentioned,  for  instance,  a  prick  on  the  sole  caused  a  reflex 
contraction  of  the  muscles  in  the  upper  parts  of  the  legs,  but  of  these 
alone.  In  many  cases,  however,  the  loss  of  sensation  has  been  abso- 
lute, and  reflex  action  also  has  been  entirely  lost.  The  tendency  to 
trophic  changes  is  great,  and  an  affection  of  the  sphincters  is  almost 
invariable.  Cystitis  and  its  consequences  readily  occur,  and  may 
reach  a  high  degree,  in  consequence  of  residual  accumulation,  when 
the  patient  is  conscious  only  of  slight  difficulty  of  micturition. 

Multiple  tumours  (met  with  especially  in  cases  of  tubercle  and 
gliomatosis)  may  give  rise  to  very  complex  symptoms.  If  there  are 
only  two  tumours,  they  may  be  indicated  by  the  successive  develop- 
ment of  focal  symptoms,  similar  in  character  but  different  in  seat. 
In  some  cases  in  which  there  are  many  growths,  only  one  or  two  of 
the  largest  have  caused  symptoms,  and  the  smaller  tumours  have 
run  a  latent  course.  In  other  cases  in  which  many  tumours  have 
caused  slight  symptoms,  these  have  simulated  closely  a  system-disease 
of  the  cord.  Thus,  in  the  remarkable  case  recorded  by  Hughes 
Bennett,  multiple  growths  on  the  posterior  nerve-roots  produced  the 
characteristic  manifestations  of  locomotor  ataxy.*  When  cerebral 
tumours  have  co-existed  with  multiple  spinal  growths,  the  symptoms 
of  the  latter  have  usually  been  lost  in  those  of  the  intra-cranial  disease 
which  has  often  preceded  the  spinal  lesion. 

The  duration  of  the  symptoms  of  tumours  of  the  cord  and  mem- 
branes, those  of  syphilitic  nature  being  excluded,  varies  from  three 
months  to  five,  or  even  ten  years.  Equally  variable,  but  shorter,  is 
the  time  that  elapses  before  the  symptoms  reach  their  full  develop- 
ment, which  depends  partly  on  the  secondary  inflammation,  partly  on 
the  rate  of  growth  of  the  tumour.  Prom  the  former  cause,  the  time 
may  be  only  a  few  days,  when  the  myelitis  is  acute ;  from  the  latter 
it  may  be  as  short  as  three  or  four  weeks,  in  tumours  of  tubercular  or 
syphilitic  nature.  In  the  majority  of  cases  death  ensues  at  the  end  of 
from  one  to  three  years  from  the  commencement  of  the  symptoms,  but 
these  last  longer  when  the  growth  is  outside  the  dura  mater  than 
when  inside  the  sheath.  The  immediate  cause  of  death  is  usually  the 
direct  or  indirect  effect  of  the  tumour ;  but  in  syphilis  death  is  some- 
•  *  Clin.  Trans.,'  1885. 


622  SPINAL    CORD. 

times  due  to  disease  elsewhere,  and  in  tubercle,  frequently  to  general 
pidmonary  tuberculosis  (which  usually  precedes  the  spinal  growth), 
or  sometimes  to  meningitis. 

Diagnosis. — The  evidence  of  a  growth  consists  in  the  symptoms  of 
a  focal  lesion  of  slow  development,  and  es]^)ecially  in  the  presence  of 
signs  of  compression  and  irritation  (pain  and  rigidity) ,  while  the  sig- 
nificance of  these  is  increased  by  indications  that  the  disease  began  at 
the  surface  of  the  cord,  and  sometimes  by  the  history  of  a  cause,  as 
syphilis.  Although  no  symptom  is  actually  pathognomonic,  yet  the 
combination  of  the  symptoms,  and  their  course,  are  in  most  cases 
sufB.ciently  characteristic.  Those  of  greatest  diagnostic  importance 
are,  first,  the  pain  that  is  felt  in  the  parts  at  the  level  of  the  tumour, 
or  below  it,  and  sometimes  in  the  spine;  secondly,  the  progressive 
paralysis,  motor  and  sensory.  Of  less  but  still  considerable  importance 
are  the  rigidity  of  the  muscles  of  the  spine,  the  muscular  contractions 
in  the  limbs,  and  the  early  and  marked  excess  of  reflex  action  when 
the  cord  itself  is  involved.  In  the  course  of  the  disease  the  most 
important  features  are  the  steady  progress  of  the  symptoms,  and 
especially  their  commencement  on  one  side  and  extension  to  the  other. 
Of  causal  indications  that  occasionally  assist  the  diagnosis,  the  most 
important  are  a  history  of  constitutional  syphilis,  the  presence  of 
tubercular  disease  elsewhere,  and,  in  rare  instances,  the  existence  in 
other  parts  of  such  multiple  growths  as  are  known  to  occur  also  within 
the  spinal  canal,  especially  multiple  sarcomata  and  neuromata. 

The  diagnosis  of  multiple  growths  depends  on  the  recognition  of  the 
successive  development  of  the  characteristic  symptoms  in  more  than 
one  focus.  It  is  rare  for  more  than  two  of  many  tumours  to  cause 
definite  symptoms.  The  greatest  absolute  difficulty  in  the  diagnosis 
of  a  spinal  tumour  is  presented  by  the  cases  sometimes  met  with,  in 
which  an  intra-cranial  tumour  precedes  the  growth  within  the  vertebral 
column,  and  the  symptoms  of  the  latter  are  overlooked  in  the  presence 
of  the  severe  manifestations  of  the  former.  In  most  cases,  however, 
the  additional  symptoms,  which  usually  exist,  may  be  detected  by 
careful  observation,  aaid  their  significance  recognised.  Growths 
involving  the  cauda  equina  cause  symptoms  like  those  of  tumours  in 
the  lumbar  enlargement,  save  that  the  effects  are  from  the  first 
bilateral  in  even  more  pronounced  degree,  and  the  evidence  of  inter- 
ference with  the  nerves  (loss  of  reflex  action,  nerve  pain,  &c.)  is  even 
more  conspicuous. 

The  differential  diagnosis  involves  the  distinction  from  diseases  of 
the  vertebral  bones,  of  the  membranes,  and  of  the  cord  itself.  Caries 
of  the  spine  has  many  symptoms  in  common  with  tumour,  but  the 
root  pains  are  rarely  very  severe,  and  the  effects  of  compression  of  the 
cord  are  usually  bilateral,  either  from  the  first  or  very  soon  after  their 
commencement ;  the  paralysis  seldom  reaches  a  considerable  degree  in 
one  leg  before  the  other  suffers.  The  signs  of  bone  disease  are  rarely 
long  absent,  and  repeated  examination  usually  reveals  tenderness  and 


TDMOUES.  623 

irregularity  of  the  vertebral  spines.  From  growths  in  tlie  bones  of 
tbe  spinal  column  the  diagnosis  may  at  first  be  impossible.  But  the 
pain  of  tumour  is  rarely  increased  by  movement  to  the  same  intense 
degree  as  when  the  growth  commences  in  the  bone,  unless  the  tumour 
is  in  the  cervical  region  of  the  cord,  and  then  the  greater  accessibility 
of  the  vertebral  column  renders  the  exclusion  of  bone  growths 
comparatively  easy  by  local  examination.  In  the  dorsal  region  a 
.  growth  in  the  bone  may  long  escape  detection,  but  even  here  the 
influence  of  movement  on  the  pain  is  often  extreme,  far  greater  than 
in  the  case  of  tumours  within  the  canal.  The  effect  of  the  movement  of 
the  vertebrae  on  the  nerve-roots,  when  compressed  as  they  pass  through 
the  foramina,  is  far  greater  than  when  they  are  compressed  within  the 
canal.  Sooner  or  later  a  local  enlargement  may  present  itself  ex- 
ternally, and  show  the  nature  of  the  case. 

"  Hypertrophic  pachymeningitis,"  affecting  the  cervical  region,  is 
the  only  meningeal  disease  which  closely  simulates  the  symptoms  of 
tumour,  but  its  effects  are  usually  from  the  first  bilateral,  and  they 
have  a  considerable  vertical  extent.  Hence  it  is  only  with  central 
tumours,  which  grow  through  several  inches  or  more  of  the  cord,  that 
the  disease  is  likely  to  be  confounded.  In  each  malady  there  may  be 
muscular  atrophy  in  the  arms,  and  paralysis,  without  wasting,  in  the 
legs.  But  in  central  tumours  the  pains  in  the  arms  are  usually  much 
slighter  than  in  the  thickening  of  the  membrane,  and  there  is  rarely 
the  early  and  limited  impairment  of  all  forms  of  sensation  which  results 
from  the  damage  to  the  nerve-roots  in  the  latter  disease.  This  is  also 
true  of  syringomyelia,  in  which  tactile  sensibility  usually  persists,  al- 
though untypical  cases  may  present  much  difficulty.  The  diagnosis  is 
considered  more  fully  in  the  account  of  that  disease. 

The  affection  of  the  cord  itself  which  most  closely  simulates  the 
symptoms  of  tumour  is  a  very  chronic  transverse  myelitis.  In  such 
a  case  as  that  shown  in  Fig.  108,  p.  383,  the  diagnosis  may  be  a  matter 
of  extreme  difficulty.  The  radiating  pains  in  this  case  were  severe 
and  local,  and  the  limitations  of  the  symptoms,  for  a  long  time,  to  one 
side,  with  crossed  sensory  and  motor  palsy,  presented  an  almost  perfect 
analogy  to  the  effects  of  a  growth.  But  it  is  very  rare  for  severe 
and  acute  radiating  pains  to  result  from  focal  myelitis,  although  a 
painful  sense  of  constriction  is  common ;  moreover,  in  tumour,  one 
half  of  the  cord  seldom  suffers  severely  without  the  functions  of  the 
other  half  also  being  very  soon  impaired  in  some  degree.  The  limitation 
of  chronic  myelitis  to  a  single  focus  is  also  very  unusual ;  a  consider- 
able vertical  extent  of  the  cord  is  usually  involved  in  varying  degree. 
Hence  the  practical  difficulty  in  diagnosis  is  not  so  great  as,  at  first 
sight,  might  appear.  Acute  or  subacute  myelitis  can  only  be  con- 
founded with  a  growth  in  cases  in  which  inflammation  results  from 
pressure,  and  develops  with  independent  energy  and  rapidity.  In 
such  cases  the  occurrence  of  myelitis  must  be  recognised,  and  the 
diagnostic  problem  is  the  detection  of  the  preceding  symptoms  of  the 


624  SPINAL  COED. 

growth  that  excites  the  inflammation.  This  is  usually  easy;  the 
severe  pains,  and  commonly  also  the  slighter  symptoms  of  paralysis, 
which  existed  before  the  rapid  increase  in  the  loss  of  power,  are  suffi- 
cient proof  of  a  chronic  morbid  process,  to  which  the  inflammation  ia 
secondary. 

The  early  pains  are  often  thought  to  be  neuralgic,  but  their  con- 
stancy and  continuance  should  suggest  organic  irritation;  their  seat, 
moreover,  is  rarely  that  in  which  neuralgia  is  common.  The  pain , 
produced  by  tumours  of  the  cauda  equina  is  often  felt  first  in  the 
sciatic  area,  and  thought  to  be  sciatica ;  but  it  is  commonly  from 
the  first  bilateral,  whilst  sciatica  is  almost  invariably  one-sided,  and 
bilateral  sciatic  pain  should  always  suggest  irritation  where  the  sen- 
sory fibres  from  both  sides  are  near  together,  and  can  be  irritated  by  a 
single  cause,  i,  e.  it  should  suggest  disease  within  the  vertebral  canal. 
In  all  cases,  however  dubious  the  early  pains  may  be,  other  symptoms 
soon  become  added,  and  prove  that  there  is  more  than  a  simple  neu- 
ralgia. But  the  effect  of  the  organic  disease  on  other  parts  of  the 
nervous  system  may  evoke  any  functional  derangement  to  which  the 
patient  is  predisposed,  and  it  is  most  important  not  to  permit  the 
diagnosis  to  be  thereby  misled,  or  the  gravity  of  organic  symptoms  to 
be  under-estimated.* 

If  the  existence  of  a  tumour  is  clear,  two  questions  remain — its  seat 
and  nature.  The  interference  with  the  elements  of  the  cord  shows 
its  lateral  seat,  but  this  is  of  small  practical  importance  compared 
with  the  evidence  of  its  vertical  position,  in  any  case  in  which  an 
operation  is  contemplated.  The  latter  is  indicated  by  the  level  of  the 
transverse  symptoms,  especially  the  upper  level  of  the  sensory  or 
motor  loss,  or  sensory  irritation;  but  it  is  of  special  importance  to 
note  that  the  growth  is  often  above  this,  and  never,  or  almost  never, 
lower.  It  may  be  even  a  distance  of  three  or  four  vertebrae  above 
the  pair  of  nerves  corresponding  to  the  highest  level  of  the  ansesthesia 
or  pain.  This  discrepancy  is  especially  noticeable  when  the  growth 
is  in  the  cord;  it  is  least  when  the  tumour  is  extra- dural;  and 
if  this  is  so  situated  as  to  affect  a  nerve  on  one  side,  there  is  often  a 
strict  correspondence  between  the  symptoias  and  the  situation  of  the 
lesion.  It  is  explained  by  the  upward  course  of  many  posterior  root- 
fibres. 

The  nature  of  the  tumour  is  also  of  great  practical  importance  in 

*  Thus  the  patient  from  whom  the  tumour  was  removed  (see  p.  627)  had  once 
had  some  convulsive  attacks,  and  the  intense  pain  due  to  the  growth  having  caused 
slight  mental  symptoms,  I  was  asked  to  see  him  to  decide  the  question  of  the 
hysterical  nature  of  the  affection.  The  fact  is  peculiarly  instructive  from  a 
diagnostic  point  of  view.  Not  less  so  is  a  case  of  central  glioma  in  a  girl  (sent 
to  me  by  Mr.  P.  B.  Mason,  of  Burton-on-Trent),  in  which  a  central  growth  in  the 
cervical  region  extended  up  into  the  medulla,  and  caused  at  last  universal  palsy, 
'I'lie  first  symptoms  immediately  followed  severe  mental  disturbance ;  to  this  they 
were  naturally  ascribed,  and  thought  to  be  functional.  A  similar  sequence  ie  met 
with  in  cerebral  tumours  {q.v.). 


TUMOURS,  625 

many  cases.  If  tlie  patient  has  liad  constitutional  syphilis  the 
syphilitic  nature  of  the  growth  is  highly  probable,  and  the  probability 
is  further  raised  in  degree  if  the  growth  developed  rapidly,  so  that  the 
symptoms  attained  a  considerable  intensity  in  less  than  three  months 
from  their  onset.  The  co-existence  of  a  cerebral  lesion  also  increases 
the  likelihood  of  the  syphilitic  nature  of  the  growth.  Either  of  the 
last  two  considerations  suggests  the  same  conclusion  in  any  case  in 
which  syphilis,  although  not  proved,  is  possible;  but  it  must  be 
remembered  that  cerebral  and  spinal  tumours,  of  other  than  syphilitic 
natm-e,  may  co-exist.  In  the  same  way,  tubercular  and  scrofulous 
processes  suggest  the  tubercular  nature  of  a  tumour.  Most  cases  of 
tubercular  growths  have  occurred  in  adults,  the  subjects  of  phthisis. 
The  diagnosis  is  made  more  probable  by  rapidity  of  course,  by  the 
speedy  extension  of  the  symptoms  to  the  second  side,  by  the  sb'ghtness 
of  evidence  of  irritation,  and,  in  some  cases,  by  periods  of  arrest  of  the 
progress  of  symptoms  that  were  slow  in  their  development.  Such 
arrest  indicates  an  arrest  of  the  growth  of  the  tumour,  which  we 
cannot  infer  from  the  subsidence  of  symptoms  that  developed  rapidly, 
and  may  have  been  due  to  secondary  inflammation.  WTien  a  rapid 
onset  is  due  to  this  cause,  irritation  symptoms  are  usually  pro- 
nounced.* Tumours  elsewhere  always  constitute  strong  evidence  of 
the  nature  of  one  in  the  cord,  but  such  an  indication  is  rarely  avail- 
able, since  the  spinal  canal  is  rarely  the  seat  of  secondary  growths. 
Hydatids  can  only  be  diagnosed  if  others  are  present  where  their 
nature  can  be  recognised ;  this  has  hardly  ever  .been  the  case  in  the  rare 
instances  in  which  such  tumours  have  existed  within  the  spinal  cord. 
Multiple  tumours  are  probably  either  tubercle,  sarcoma  or  neuroma. 
In  the  absence  of  these  indications  a  meningeal  tumour  is  probably  a 
sarcoma,  perhaps  fibrous  or  myxomatous,  and  one  within  the  cord  is 
probably  a  glioma  or  tubercular,  because  these  are  the  most  frequent 
growths  in  those  situations.  A  diagnosis  founded  on  mere  frequency 
of  occui-rence  has  necessarily  only  a  low  degree  of  probability,  and 
must  be  sometimes  wrong;  but  it  may  be  highly  probable  that  the 
growth  is  one  of  two  or  three  kinds,  and  this  may  be  of  considerable 
practical  value.     To  the  surgeon  the  point  is  of  great  importance. 

Peognosis. — ^Unless  the  tumour  is  syphilitic,  or  can  be  removed, 
the  prognosis  is  necessarily  most  grave.  Of  all  syphilitic  lesions, 
growths  are  those  that  are  most  amenable  to  treatment,  and  the 
effects  of  which  most  certainly  pass  away  if  they  have  not  reached 
an  extreme  degree  or  lasted  too  long  a  time.  If,  however,  the  damage 
has  been  allowed  to  remain  unchecked  for  several  months,  recovery 

*  Thus  numbness  in  the  left  leg,  with  increased  sensitiveness  to  pain  in  the 
thigh,  WEB  quickly  followed  by  complete  paraplegia;  the  hypersesthesia  became 
restricted  to  the  region  supplied  by  the  first  lumbar  nerves.  Death  occurred  two 
months  after  the  onset  of  the  symptoms,  and  a  tubercular  tumour  was  found  in  the 
upper  part  of  the  lumbar  enlargement,  occupying  almost  the  whole  thickness  of  tho 
cord  (Ludeck,  *  Jahrb.  d.  Hamburg.  Staatskr.,'  1896). 

VOL.  I.  40 


626  SPINAL    CORD, 

may  be  imperfect.  Tumours  of  most  other  kinds  steadily  increase,  and 
the  resulting  damage  is  on  tlie  wliole  steadily  progressive,  althougli  its 
course  may  be  varied  by  stages  of  rapid  (myelitic)  increase  in  the 
symptoms,  followed  by  stationary  periods  and  even  by  transient 
improvement.  In  tbe  case  of  growths  outside  the  cord  the  prognosis 
has  been  rendered  much  less  gloomy  by  the  progress  of  surgery. 

Tkeatment. — If  the  growth  is  syphilitic,  appropriate  treatment 
should  be  energetic  and  prompt,  so  as  to  effect  some  diminution  of 
the  pressure  as  quickly  as  possible,  since  the  longer  this  continues 
the  more  considerable  are  the  degenerative  changes,  and  the  longer 
these  will  endure.  Even  the  influence  of  a  few  days  before  treatment 
becomes  effective  may  make  a  difference  of  weeks  in  the  duration  of 
symptoms,  and  lessen  the  degree  of  ultimate  recovery.  Whenever 
the  symptoms  suggest  a  tumour,  and  syphilis  is  possible,  it  is  most 
important  that  suitable  treatment  should  be  employed ;  it  can  do  no 
harm  if  the  tumour  is  not  syphilitic,  and  may  save  the  patient's  life 
and  strength  if  it  is.  Syphilis  can  only  be  excluded  when  there  has 
been  no  possibility  of  infection.  In  many  cases  of  late  syphilitic 
lesions  there  is  no  history  of  secondary  syphilis,  and  in  others  there  is 
no  history  of  a  primary  sore.  Hence  it  is  a  priori  certain  that  in  some 
cases  of  late  lesions  a  history  of  both  primary  sore  and  secondary 
symptoms  will  be  absent,  and,  as  a  matter  of  fact,  such  cases  are  met 
with  not  unfrequently.  Of  course  these  cases  cannot  be  counted  as 
syphilitic  in  any  scientific  investigation  into  the  influence  of  this 
disorder,  but  in  treatment  we  have  often  to  allow  weight  to  considera- 
tions which  are  insufficient  to  influence  scientific  investigation,  and 
treatment  determined  by  the  mere  possibility  of  syphilis  is  often 
justified  by  its  results.  Tubercular  tumours  are  also  amenable  to 
treatment  to  some  extent,  but  they  are  too  rare  to  be  of  much  practical 
importance. 

In  most  other  cases  we  can  do  little  more  than  treat  symptoms, 
relieving  pain  by  sedatives,  watching  the  state  of  the  bladder, 
preventing  the  occurrence  of  cystitis,  and  guarding  the  patient 
from  bedsores.  We  must  remember  that  sedatives  will  probably  be 
needed  for  a  long  time,  and  they  must  therefore  be  used  as  sparingly 
as  possible,  lest  custom  and  tolerance  deprive  them  of  their  power. 
Cocaine  may  be  used  in  many  cases  as  an  aid  in  economising  the 
influence  of  morphia. 

But  many  tumours  within  the  spinal  canal  may  be  removed,  at  any 
rate  if  they  are  outside  the  substance  of  the  cord.  The  anticipation 
that  such  growths  would  be  found  removable  was  expressed  in  the  first 
edition  of  this  book  (1886),  in  words  that  may  be  quoted  because 
they  were  soon  afterwards  realised: — "Modern  methods  render  the 
opening  of  the  spinal  canal  far  less  formidable  than  it  formerly  was, 
and  the  removal  of  a  tumour  from  the  membranes  of  the  cord  would 
involve  less  immediate  danger  of  serious  consequences  than  the 
removal  of  a  tumour  from  the  brain." 


TUMOUliS.  627 

On  tlie  other  hand,  growths  witliin  the  spinal  cord  are  in  a  very 
different  position  with  regard  to  sui-gical  procedure,  since  the  removal 
of  such  a  tumour  could  hardly  fail  to  cause  a  traumatic  inflammation 
that  would  damage  and  perhaps  destroy  all  the  elements  of  the  cord  at 
the  spot,  and  would  probably  be  permanent  in  its  effects,  especially 
when  these  structures  have  already  suffered  from  pressure.  At  the 
same  time  the  early  removal  of  a  small  growth  might  possibly  be 
followed  by  the  regeneration  of  conducting  fibres  that  are  on  the  other 
side  of  the  organ,  and  the  return  of  their  f "unction,  lost  only  through 
the  effects  of  pressure. 

In  a  man  with  complete  paraplegia,  motor  and  sensory,  of  slow 
development,  accompanied  by  attacks  of  agonising  spasm,  a  diagnosis 
of  tumour  led  me  to  advise  the  removal  of  the  growth.  This  was  done 
by  Mr.  Horsley,  and  the  result  has  been  the  permanent  recovery  of  the 
patient.  Although  the  intense  spastic  paraplegia  showed  that  there 
must  have  been  complete  degeneration  of  the  pyramidal  fibres,  every 
indication  of  this  has  passed  away.  The  tumour  was  a  myxofibroma, 
about  the  size  of  a  spht  almond,  which  had  so  compressed  the  cord  in 
the  upper  dorsal  region  as  to  reduce  its  thictness  to  about  one  half.  It 
was  within  the  dural  sheath,  and  adherent  to  the  cord.*  It  is 
certain  that  many  growths  might  be  removed  without  difficulty,  and 
with  a  secure  result,  although  not  many  instances  have  been  since 
recorded. 

In  connection  with  an  operation,  a  question  sometimes  arises  that 
is  of  very  great  importance,  and  often  of  equal  difficulty.  The  sym- 
ptoms are  consistent  with  the  syphilitic  nature  of  the  growth ;  and  this 
cause  and  nature  cannot  be  excluded,  except  by  the  disproof  afforded 
by  the  inutility  of  treatment.  But  it  is  essential  that  this  disproof 
should  be  afforded  before  an  operation  is  decided  on;  and  the  question 
arises,  how  long  should  be  allowed  for  this  process  of  practical  exclusion, 
— how  long  should  treatment  be  continued  without  result  to  justify 
the  last  resort  ?  It  needs  to  be  longer  when  the  symptoms  are  of  con- 
siderable duration  than  when  they  are  recent,  since  secondary  changes 
of  long  duration  cannot  be  qmckly  removed.  Time  is  needed  in  every 
instance  to  permit  a  trustworthy  conclusion,  but  a  month  is  pro- 
bably always  enough  to  enable  a  result  to  be  reached  that  wiU  justify 
either  further  delay  on  the  one  hand,  or  immediate  procedure  on  the 
other. 

Finally,  one  injunction  given  by  Mr.  Horsley  may  be  emphatically 
endorsed.  If  it  is  clear  that  the  growth  is  not  syphilitic,  and  that  no 
good  can  be  done  by  other  treatment,  delay  in  an  operation  can  only 
cause  harm — can  only  result  in  a  less  favourable  state  for  the  proceed- 

•  The  details  of  the  operation  are  full  of  insfcraction  in  reference  to  similar 
procedures,  and  are  given  in  the  account  of  the  case  in  the  '  Med.-Chir.  Trans.,' 
vol.  Ixii,  1888.  TLe  surgical  aspect  of  the  subject  is  too  large  to  be  entered  ou 
here. 


628  SPINAL  COED. 

ing,  less  chance  of  recovery,  less  degree  of  recovery,  longer  and  greater 
«ufiering,  and  sliould  on  every  ground  be  avoided. 


OAYITtES  AKD  FISSURES  IK  THE   SPINAL  COED 

(STEINGOMTEIilA,  HTDBOMTELIA,  HTDEOEEHACHIS  INTEKNA).* 

Cavities  in  the  spinal  cord,  distended  with  liquid,  are  met  with  at 
all  ages,  and  those  which  present  certain  features,  and  often  cause 
certain  symptoms,  are  now  known  by  the  above  terms.  The  following 
figures  illustrate  the  most  important  facts  regarding  this  morbid  state, 
and  its  somewhat  complex  conditions  will  be  best  understood  by  con- 
sidering the  appearances  presented  in  these,  and  their  significance, 
as  described  in  the  explanations. 

The  term  "Syringomyelia"  is  applied  to  all  cavities  in  the  cord 
with  well-defined  limits ;  but  the  majority  of  these  (with  the  exception 
of  such  as  exist  in  tumours  of  manifestly  different  nature),  whatever 
their  condition  or  form,  or  the  changes  they  have  undergone,  are  sur- 
rounded by  a  layer  of  embryonal  neuroglial  tissue,  and  outside  this 
the  white  substance  of  the  cord  is  deficient  in  amount.  Wherever 
met  with,  or  whatever  the  secondary  changes  the  tissue  presents,  this 
fact  of  environment  stamps  the  cavity  or  cavities  (for  there  may  be 
two)  as  congenital,  because  this  abnormal  material  is  due  to  a  per- 
sistence of  the  embryonal  tissue  from  which  the  cord  is  developed. 
The  cavities  are  thus  due  partly  to  defective  closure  of  the  tube  which 
is  first  formed  (residual  portions  of  which  become  distended  by 
accumulation  of  liquid),  and  partly  to  the  breaking  down  of  this  per- 
sistent embryonal  tissue  or  to  tissue  formed  from  it  by  a  process  of 
growth.  Moreover  the  cord  sometimes  suffers  serious  damage  from 
pressure,  owing  to  the  distension  of  the  cavity  by  the  liquid  which 
accumulates  within  it,  or  by  growth  of  the  adjacent  tissue.  The  con- 
dition is  not  rare,  but  is  often  overlooked,  especially  in  its  slighter 
forms,  and  is  readily  misinterpreted  when  it  is  associated  with  acquired 
disease — as  in  the  condition  illustrated  in  Pig.  109,  p.  385,  which  is 
very  instructive  in  this  connection.  The  term  "  Hydromyelia "  has 
been  applied  to  the  simpler  f  orms,t  in  which  the  cavity  is  merely  the 

•  During  the  last  few  years  many  cases  of  this  disorder,  in  which  the  condition 
was  verified  by  post-mortem  examination,  have  been  published  in  this  country,  as 
well  H8  in  France,  Germany,  and  America.  Several  monographs  have  also  appeared, 
the  most  noteworthy  being  those  of  Hoffmann  (*  Deutscii.  Ztsch.  f .  Nervenheilk.,* 
1892)  and  Schlesinger  ("  Die  Syringomyelia,"  '  Leipzig  and  Wien,  Franz  Deuticke,* 
1895).  To  these  the  reader  is  referred  for  more  detailed  information  on  this 
interesting  disease. 

t  See  Leyden,  •  Virchow's  Archiv.'  Bd.  Izviii,  p.  1.    Hofifmann  (loc.  cit.)  divide:^ 


SYRINGOMYELIA.  629 

dilated  central  canal ;  but  as  there  is  no  real  difference  between  this 
and  other  varieties,  the  designation,  being  unnecessary  and  even  mis- 
leading, is  falling  into  disuse. 

To   understand   the  origin  of   these  conditions  it  is  necessary  to 
remember  the  mode  of  development  of  the  spinal  cord.     The  sides  of 
the  primitive  furrow  of  the  embryo  coalesce,  so  as  to  form  a  canal  of 
relatively    large  size,    the   walls  of   which  are  thinner  in  front  and 
behind  than  at  the  sides,  and  consist  of  elongated  cells,  some  of  which 
are   arranged  regularly   at  the  inner  surface  of  the  cavity,  like  an 
epithelium.     The  anterior  wall  first  becomes  thickened,  to  form  the 
wliite  commissure  and  the  front  part  of  the  grey,  while  an  abundant 
growth  of  cells  occurs  at  the  sides  of  the  tube,  and  extends  forwards 
(i.  e.  downwards  in  the  position  of  the  embryo),   thus  forming  the 
lateral  and  anterior  columns  ;  between  these  two   projections  is  the 
depression  of  the  anterior  fissure.     Next  occurs  a  growth  backwards 
of   the   now   thick    sides,  which   forms    the    posterior   parts  of   the 
lateral  columns  and  the  adjacent  part  of  the  posterior  columns,  and 
with   this  the  posterior  roots  are  connected ;  this  forms  the  "  root- 
zone  "  of  the  posterior  columns.      The  posterior  median  columns  are 
formed  later.     The  central   canal  extends  backwards  between  these 
two  rudimentary  postero-external  columns,  being  closed  behind  only 
by  a  thin  layer  of  cells.     As  the  posterior  columns  increase  in  thick- 
ness the  posterior  part  of  the  canal  becomes  narrowed,  and  its  walls 
unite  near  its  anterior   extremity  so  as  to  form   the   posterior  grey 
commissure,  and  divide  the  canal  into  two  parts.     Of  these  the  ante- 
rior becomes  the  permanent  canal,  while  the  posterior  is  reduced  to  a 
narrow  fissure  between  the  posterior  columns.     Ultimately  this  poste- 
rior narrow  part  becomes  closed  by  a  growth  of  cells  which  occurs 
from  behind  forwards,  as  the  median  parts  of  the  posterior  columns 
develop.      All  these  parts    consist    first  of    embryonal    cells,   which 
afterwards  undergo  a  transformation  into  the  nerve-elements  proper, 
and  the  order  of  transformation  is  the  same  as  that  in  which  the 
parts  were  formed,  the  posterior  median  columns  latest.     The  cells  of 
the  grey  matter  are  formed  before  the  fibres  of  the  white  columns. 
Some  of  the  embryonal  elements  undergo   a   different  and  slighter 
change.       They   may   almost   be  said   to    persist,    constituting,   the 
neuroglia,    and  the   gelatinous    grey   substance   continuous   with    it 
around  the  cord,  and  also  massed  at  the  posterior  root  fissure  and 
around  the  central  canal. 

The  central  canal  ultimately  lies  in  the  anterior  part  or  middle  of 
the  grey  commissure.  It  may  persist  through  life  as  a  cavity,  cir- 
cular in  section,  or  as  a  slit,  antero-posterior  or  transverse,  and  is 
lined  with  epithelium    (Fig.    183).       Around   the   canal   there   are 

the  cases  as  follows: — I.  Hydromyelus,  which  runs  a  latent  course  without  recoa:- 
nisable  symptoms.  II o.  Primary  (central)  gliosis,  with  or  without  hydromyelus: 
(1)  without  cavity  formation;  (2)  with  splits  and  cavity  formation.  115.  Central 
gliomatosis,  with  or  without  cavity  formation. 


630  SPINAL   COED. 

usually  many  nuclear  elements,  especially  on  eacli  side  of  it,  and 
these  frequently  fill  up  the  lumen  of  the  canal,  iso  that  its  position  is 
indicated  only  by  an  OYal  mass  of  small  cellular  elements  (Fig.  183,  d). 
The  obliteration  of  the  canal  often  occurs  quite  early  in  life,  and  is  no 
evidence  of  any  morbid  process.  The  canal  may  be  found  obliterated 
in  one  part  of  the  cord,  and  patent  in  another  (c,  D,  Fig.  183),  The 
cimple  closure  of  the  canal  by  nuclei  does  not  usually  cause  any  disten- 
sion of  the  part  which  is  above  the  closed  portion,  or,  at  any  rate,  not 
more  than  a  very  slight  increase  in  size,  so  trifling  that  its  signifi- 
cance is  open  to  question. 

Syringomyelia    occurs    cliiefly  in  the 

^  .  cervical  and  upper  dorsal  regions  of  the 

'^^'  cord.     The  cavity  is  often  closed  below 

'V         A  by  gliomatous  tissue,  and  the  excess  of 

this  may  extend  up  into  the  medulla  and 

even  jDons.    Occasionally  the  cavity  exists 

^£  through  the  whole  length  of  the  cord, 

'3.,  and  may  extend  into  the  medulla  oblon- 

"\;/  gata,  and  then  affect  the  aqueduct  of 

Sylvius  and  the  tissue  around  it. 
r  ■■ .  /  ■  In  the  simple  condition  found  in  young 

y  children,  or  in   adults  when  no   secon- 

\;  dary  changes  have  occurred,  the  morbid 

change  presents  the  two  features  already 
mentioned — a  quantity  of  tissue  of  trans- 
lucent aspect,  which,  from  its  structure, 
t  is  clearly  persistent  embryonal  tissue  in 

%_  ~,     which    nerve-elements    have    not    deve- 

"  t  >  loped,  and  a  cavity  due  to  imperfect  con- 

^  traction  of  the  canal.      The  change  is 

Pia.    183.-Central     canal     in  ^^^        confined  to  the  posterior  half  of 

normal  cords.  It  has  the  rorm  or  ''  -"^ 

a  transverse  slit  in  A,  a  vertical  the  cord,  and  usually  to  the  postenor 
slit  in  C  (from  the  conus  medul-  columns,  which,  as  we  have  seen,  are 
laris),  while  in  B  it  is  circular.    »  i   i     j.       xx  i  t. 

In  each  it  is  lined  by  columnar  formed  last.  It  may,  however,  be  pre- 
epithelium  and  surrounded  by  sent  in  one  or  both  posterior  horns,  in 
cellular  elements,  rounded,  or  ^j^gj^  cavities  also  may  be  formed.     The 

angular      trom      compression,  .  .  i  i    ";• 

mingled  with  granules.  In  D,  persistent  embryonal  tissue  may  be  evi- 
wbich  is  from  the  same  spinal  dently  increased  by  a  process  of  various 
TntlT.  ^;c!;;irbf.°l'Bsol  degrees  of  growth,  and  the  cavity  enlarged 
nuclear  tissue.  by  distension.    Further,  when  the  persis- 

tent tissue  is  considerable  in  quantity  it 
may  break  down,  and  thus  give  rise  to  a  new  cavity,  or  enlarge  that 
which  has  developed  from  the  primitive  canal.  Thus  the  cavity  may  be 
distinct  from  the  canal,  and  in  some  cases  the  abnormal  tissue  around 
it  is  very  small  in  amount,  and  reduced  even  to  a  mere  wall.  It  may 
also  be  separate,  because  it  is  the  hinder  part  of  the  embryonal  canal, 
cut  off  by  coalescence  of  the  walls. 


SYKINGOMYELTA.  631 

These  conditions  are  shown  very  well  in  the  adjoining  figures,  after 
Leyden  (Figs.  184,  185).  In  the  first,  at  a,  the  cavity  presents 
nearly  the  shape  which  it  has  at  one  period  of  development,  before 
the  formation  of   the  posterior  columns  is   completed,  and  without 


Fig.  184. — Syrln<romyelia,  from  a  child  two  years  old,  with  encephalonele  :md 
absence  of  cerebellum.  (After  Leyden.)  In  A,  cervical  region,  the  central  canal 
is  large,  lined  with  epithelium,  and  from  it  a  medial  fissure  extends  backwMrds 
nearly  to  the  periphery  of  tlie  cord,  limited  by  a  layer  of  homogeneous  tissue, 
more  abumlant  at  the  posterior  limit.  In  B,  lower  down,  the  fissure  is  enhirged 
to  a  cavity  of  considerable  size,  while  in  C  it  is  still  larger,  and  the  posterior 
columns  are  reduced  to  a  narrow  zone  between  the  layer  of  tissue  which  bounds 
the  cavity  and  the  grey  suhst:ince. 

the  separation  into  two  parts  produced  by  the  formation  of  the  pos- 
terior commissure.  It  is  bounded  by  a  narrow  layer  of  embryonal 
tissue,  which  widens  out  behind,  having  nearly  the  shape  of  the  pos- 
terior median  columns,  which  are,  as  we  have  seen,  the  last  to  be 
formed.  In  b  and  c  the  cavity  is  much  larger,  and  the  posterior 
columns  are  smaller,  either  by  an  earlier  arrest  of  development  or  by 
greater  distension,  or,  most  probably,  by  the  influence  of  both  these 
agencies.  The  cavity  extends  up  to  the  posterior  surface  of  the  cord, 
and  is  closed  in  there  only  by  a  narrow  layer  of  cellular  structure. 

In  Fig.  185  we  have  somewhat  more  complex  conditions.  The 
cavity  in  the  first  section  closely  resembles  in  appearance  that  in  A  of 
the  last  figure,  but  differs  in  the  important  fact  that  the  posterior  com- 
missure has  apparently  been  formed  and  the  central  canal  developed 
in  the  normal  manner,  although  it  has  become  obhterated  by  nuclei. 
This  is  clear  if  the  commissure  is  compared  in  the  several  figures. 
The  cavity,  therefore,  does  not,  as  in  Fig.  184,  represent  the  whole  of 
the  primitive  canal,  but  only  the  posterior  portion  after  its  division. 
The  adjacent  tissue  is  much  more  abundant  than  in  the  other  case. 
The  projection  into  the  posterior  extremity  of  the  cavity  indicates  a 
process  of  active  growth,  which  is  also  shown  by  the  irregularity  of 
the  cavity  in  b,  the  large  amount  of  tissue,  and  the  wide  separation  of 
the  posterior  horns.  In  c,  from  the  lower  part  of  the  cord,  the 
embryonal  tissue  occupies  two  oval  areas  in  the  posterior  column  (by 
mistake,  clear  in  the  figure),  while  the  cavity  is  closed  or  nearly  so. 
(Lower  down,  a  cavity  existed  in  the  centre  of  each  of  these  areas, 
apparently  due  to  breaking  down  of  the  tissue.)  In  d,  again,  the 
embryonal  tissue  occupies  only  the  middle  line,  as  a  wedge-shaped 
area,  due  to  the  defective  formation  of  the  medial  part  of  the  posterior 
columns.  It  has  been  mentioned  that  the  neuroglia  may  be  regarded 
as  a  persistent,  slightly  modified  embryonal  tissue,  and  these  exuberant 


632 


SPINAL   COED. 


^ 


masses  of  tissue  "bear  considerable  resemblance  in  structure  to  gliomata, 
so  that  the  condition  lias  been  called  "  gliomatosis."     In  eacb  of  these 

— =— —  cases  the  brain  participated  in  the  defect  of 

\    development;    there    was    internal   hydro- 
'   cephalus,  and  the  cerebellum  was  absent. 
The  cavities  in  the  adult  cord  present 
/     many  varieties.     The  most  common  form 
resembles  in  its  essential  features  the  con- 
_  ^  A      genital   disease   which   we   have  just   con- 

sidered. Between  the  two  there  are  also 
,^''<^T^^>/  ^--^  ^  differences,  but  the  resemblance  is  suffi- 
ciently close,  and  the  origin  of  the  differ- 
ences is  sufficiently  clear,  to  make  it  prac- 
tically certain,  as  already  mentioned,  that 
the  two  forms  are  really  the  same,  and 
that  the  form  met  with  in  adults  is  the 
congenital  condition,  persisting  until  later 
life.  Of  the  characters  which  this  form  has 
in  common  with  the  congenital  disease,  the 
most  important  are  the  position  of  the  cavi- 
ties and  the  presence  of  tissue  in  their 
vicinity  of  embryonal  or  gHomatous  nature. 
The  differences  depend  on  an  apparent 
/^  \  increase   of    this    tissue  by  a    process    oi 

/  t  growth,  on  the  greater  damage  to  the  other 

|,  J  parts  of  the  cord  by  the  distension  of  the 

\(  cavity,  and  on  the  extent  to  which  the  for- 

^«-  U  mation  of  new  cavities  or  enlargement  of 

Fn*.    185.  —  Synngomyelia,  old  ones  takes  place  by  a  process  of  disin- 
from  a  child  nge.l  two  and  a  tegration  of  the  newly  formed  or  persistent 

hiiltyears,  with  internal  liy-      .^  "^  -^  _ 

drocephalus  and  absence  of  tissue.     The  damage  to  the  cord  produced 
cerebellum.  (After  Leydeii,  by  distension  of  the  cavity  involves  most 

IxTiig^'A!  B.^cervical^e-  *^®  ^^^^®^  ^'^^   ^^^^  resisting  parts.     The 
gion ;  gelatinous  (embryo-  grey  substance,  therefore,  suffers  in  greatest 

Qal)  tissue  in  the  posterior  degree ;    the   white   columns   to   an   extent 
eolumns  encloses  a  medial     ,  . 

cavity,  lined  in  places  with  that  depends  m  part  on  the  precise  posi- 
cylindrical  epithelium,  and  tion  of  the  cavities,  as  presently  to  be  de- 
l:t'tt'?:S:„7''S!  -rfted.  me  kte  aI.o  present,  ia  many 
lower  dorsal  j  in  each  poste-  cases,  the  complicating  changes  of  second- 
rior  column  IS  a  mass  of  ^^^  degeneration,  ascending  or  descending, 
similar  gelatinous  substance  •'  ,  °  .  —..  nn,^  i  j  ji 
(which,  by  a  mistake,  is  as  shown  m  Eig.  186,  due  to  the  com- 
shMwn  in  C   as   an   open  pression  of  certain  tracts  at  some  specially 

cavity).         In    D,    lumbar    „f|:„„x    n  . 

region,  this  tissue  occupies   ^-nectea  spot. 

only  the  position  of  the  pos-       As    the    simplest    condition,    we    maj 

terior  median  columns.  -^^^^    ^    dilatation    of    the    Central    canal 

surrounded  by  gliomatous  tissue  disposed  as  in  congenital  cases. 
This   tissue   is   often   more   abundant,    and  forms   a  more  distinct 


SYEINGOMYELIA. 


633 


mass,   lower    down  the  cord, 
below    the    cavity.      A   good 
example  of  tliis   condition   in 
slight  degree  is  presented  b\ 
the  cord,  the  seat  of  chronic 
myelitis,    represented   at   Fig. 
109,   p.  385.     In  the  cervical 
region    (a)    there   is   a   large 
central  canal,    bounded  by  a 
thick     layer     of     gliomatous 
tissue.     In  the  inner  part  of 
this  tissue,  limiting  the  cavity, 
is  a  sinuous  membrane,  fibrous 
in  structure.      The  origin   of 
this  membrane  is  not  easy  to 
explain,  but  it  is  often  found 
in   cavities   of  this   character, 
and  is  evidence  of  the  similar 
nature    of    those    that    differ 
much   in  other   respects.    In 
the    lumbar    region     (b)    the 
tissue,  which  bounds  the  cavity 
ia  the  other  section,  forms  a 
large  round  mass  in  the  posi- 
tion of  the  canal,  obliterating  it. 
When  a  central  cavity  ap- 
pears not  to  be  the  canal  itself, 
but  to  be  situated  behind,  it  is 
then  apparently  often  due  to  a 
persistence  of  the  fore-part  of 
t^s  posterior    portion  of    the 
canal,  after  the  posterior  com- 


FlG.  186. —  Syringomyelia,  from  a 
case  of  tumours  of  the  pons  and 
Cauda    equina,    with    some     diffuse  ^^  '^ 

Bcleiosis   in    the    dorsal    region    of  *%v' 

the  cord.     A,  B,  and  C  are  sections  "^^  ' 

of   the  cervical    enlargement.      The  -il--''  'Z' 

large  cavity   iu    A    is   perhaps   the  •%;^-^- 

dilated   canal,   since   it    is   lined   in  W/  £• 

front  with  epithelium,  but  in  front  of  it,  in  the  posterior  commissure,  is  a  group  of 
nuclei  like  an  obliterated  canal.  The  zone  of  tissue  around  the  cyst  is  composed  of 
interlacing  fihres  and  nuclei,  and  processes  extend  from  it  into  the  posterior 
columns  both  in  front  and  behind.  In  B  the  cavity  is  smaller  and  the  wall 
thicker,  while  in  C  the  tissue  about  it  forms  a  considerable  mass,  and  a  second  small 
cavity  has  formed  in  the  middle  line,  apparently  by  the  brenking  down  of  the  tissue. 
In  the  dorsal  region  (D)  there  was  merely  an  unusually  large  accumulation  of  nuclei 
in  the  position  of  the  canal,  which,  in  the  lumbar  region,  bad  the  normal  aspect  (E)." 
The  cord  presents  degeneration  of  the  right  pyramidal  tract  (descending  from  the 
tnmour  in  the  pons),  and  of  the  post.  med.  cols,  and  ant.  lat.  ascending  tract,  pro- 
bably ascending  from  the  sclerosis. 


634  SPINAL    COED. 

missure  has  been  formed.  In  Fig.  186,  for  instance,  we  have  a 
central  cavity,  but  in  the  commissure  in  front  of  this  is  a  mass  of 
nuclei  having  the  usual  aspect  of  an  obliterated  canal.*  The  cavity 
is  surrounded  by  a  zone  of  tissue,  which  in  a  and  b  sends  off  a  fringe 
of  processes,  especially  backwards.  This  tissue  is  increased  in  the 
lower  part  of  the  cervical  region  (c) .  In  b  a  small  cavity  is  formed 
between  the  tissue  and  the  grey  matter  by  breaking  down,  and  in  c 
another  exists  in  the  middle  line  behind  the  growth,  probably  due  to 
deficient  closure  of  the  original  fissure,  and  increased  by  a  destructive 
process,  Still  lower,  the  commissure  resumes  its  normal  appearance, 
but  the  collection  of  nuclei  in  the  position  of  the  canal  is  unusually 
large  (d).  In  the  lumbar  region  the  canal  is  patent  (e).  The 
degeneration  of  the  posterior  median  column  and  of  the  lateral 
columns  is  secondary. 

We  have  seen  that  in  the  congenital  cases  we  must  recognise  a 
process  of  growth  of  the  persistent  embryonal  tissue  to  account  for 
some  of  the  conditions  met  with,  and  a  like  process  is  suggested  by 
the  central  mass  in  Fig.  109,  b.  Hence  it  is  not  surprising  that  in. 
many  cases  the  growth  should  attain  the  dimensions,  and  assume  the 
characters,  of  a  positive  tumour.  In  most  cases  the  tumour  has  been 
central  in  position,  and  has  had  the  structure  of  a  glioma.  It  has 
occupied  a  large  part  of  the  area  of  the  cord  at  a  certain  level.f  In 
the  chapter  on  the  tumours  of  the  cord  it  has  been  pointed  out  that 
sarcomata  also  may  grow  from  the  tissue  around  the  central  canal,  as 
in  the  case  shown  in  Fig.  181.  Hence  it  is  not  surprising  that  the 
condition  of  syringomyelia,  even  if  this  is  congenital  in  origin,  should 
be  frequently  associated  with  definite  tumours.  The  growths  have 
often  been  multiple,  apparently  the  result  of  a  wide-spread  tendency,  and 
perhaps  connected  with  a  wide-spread  persistence  of  embryonal  tissue, 
of  which  remarkable  instances  are  sometimes  met  with.  In  the  case 
shown  in  Fig.  186  there  was  a  tumour  of  the  pons  and  also  one  of  the 
cauda  equina.  The  nature  of  the  growths  is  uncertain,  but  they  were 
probably  either  sarcoma  or  glioma.  The  same  tendency  to  morbid 
growths  is  illustrated  also  by  Fig.  187,  which  is  similar  in  many  respects 
to  that  just  considered.  In  this  case  also  there  was  a  tumour  of  the 
pons  and  one  of  the  cauda  equina,  and  there  was  also  a  central 
growth  in  the  dorsal  region,  occupying  the  greater  part  of  the  area  of 

*  This  is  the  probable  interpretation.  At  the  same  time  it  is  possible  that  the 
auclei  have  not  this  significance,  and  that  it  is  really  the  central  canal.  It  is  often 
more  ditficult  than  might  be  imagined  to  say  whether  a  cavity  does  or  does  not 
represent  the  central  canal.  The  presence  or  absence  of  an  epithelial  lining  has 
generally  been  taken  as  a  criterion,  but  it  is  doubtful  whether  this  has  any  signifi- 
cance. Around  the  whole  of  the  original  cavity  the  inner  layer  of  cells  is  arranged 
as  an  epithelium,  and  if  any  part  of  it  persists  it  is  probable  that  epithelium  will 
persist  also.  Moreover  the  epithelium  often  disappi  ars  from  the  wall  of  the  dilated 
central  canal  itself;  always  where  it  is  enlarged  by  breaking  down  of  tissue. 

f  As  in  an  interesting  case  described  and  figured  by  Riesinger,  'Virchow'a 
Archiv,'  Bil.  xcviii. 


SYRINGOMYELIA. 


635 


^ 


the  cord,  to  wliich  the  ascending  degenerations  were  secondary.  In  a 
there  is  a  large  central  cavity  which  is  probably  not  the  ceutr.J  canal, 
since,  as  in  the  last  figure, 
the  position  of  this  is  marked 
by  the  oval  group  of  nuclei 
in  the  anterior  part  of  the 
grey  commissure.  The  zone 
of  tissue  which  bounds  it  is  i 
narrow,  but  a  few  processes 
are  given  off  behind.  In  b, 
first  dorsal,  this  tissue  forms 
a  compact  mass,  and  the  pro- 
cesses are  numerous  and  very 
similar  to  those  in  the  last 
figure.  In  c  the  commissure 
is  reduced  almost  to  normal 
conditions.  In  this  cord  we 
have  also  an  instance  of  the 
fact  that  cavities  may  occur 
in  other  parts,  very  similar 
in  their  general  characters  to 
those  that  occur  in  the  central 
region.  In  b  a  large  cavity 
occupies  the  intermediate 
grey  matter  and  posterior 
cornu  on  the  right  side,  with 
some  morbid  tissue  bounding 
it  on  the  medial  side.  In 
c     the     cavity     is     smaller 

Fig.  187. — Syringomyelia,  from  a  case  in  which  there  were  tumours  in  the  dorsal 
region  of  tlie  cord,  the  camla  equina,  and  the  pons  Varolii.  A,  mid-cervical  region. 
A  large  oval  cavity  lies  behind  the  grey  commissure,  surrounded  by  a  narrow  wall, 
chiefly  composed  of  fine  fibres,  but  with  a  wavy  membrane  on  the  inner  surface. 
In  front  of  the  posterior  commissure,  an  oval  group  of  nuclei  has  the  aspect  of 
an  obliterated  central  canal.  A  few  short  processes  extend  from  the  wall  of  the 
cavity  into  the  posterior  columns.  There  is  secondary  ascendino:  degeneration  of 
the  post.  med.  cols.,  right  direct  pyramidal  tract,  and  ascending  antero-lateral 
tract  in  all  the  sections.  B,  first  dorsal.  Behind  the  posterior  commissure  is  a 
growth  consisting  of  small  cells, round  and  fusiform, the  latter  with  round  nuclei. 
It  contains  many  vessels  with  thickened  walls.  A  fringe  of  fine  processes  extends 
trom  it  into  tlie  posterior  columns.  An  irregular  cavity  occupies,  on  the  right 
side,  tlie  posterior  half  of  the  grey  matter,  which  is  reduced  to  a  narrow  layer 
around  the  cyst.  This  is  lined  by  a  delicate  nucleated  membrane,  outside  which 
is  a  thicker  membrane,  lying  in  folds,  the  section  of  which  is  thus  sinuous.  Out- 
side this  again  is  a  small-celled  growth  similar  to  that  behind  the  commissure. 
In  C,  a  little  lower,  this  cavity  is  smaller,  the  sinuosities  of  the  wall  greater,  and 
the  growth  outside  it  more  abundant.  (Still  lower  the  area  was  entirely  occupied 
by  the  growth.)  In  the  posterior  commissure  there  is  only  a  quantity  of  loose 
nucleated  tissue  in  the  situation  of  the  growth.* 

*  For  the  opportunity  of  drawing  these  sections  I  am  indebted  to  Dr.  Dreschfeld, 
A  full  account  of  the  case  has  been  published  by  Dr.  Harris  in  'Brain,*  Jan.,  1886. 

The  large  growths  were  sarcomata. 


636 


SPINAL  CORD. 


and  the  tissue  around  it  is  more 
abundant,  but  easily  distinguish- 
able from  the  grey  substance, 
while  a  little  lower  down  the 
cavity  disappeared  and  the 
tissue  formed  a  compact  rounded 
mass.  The  cavity  is  lined  by  a 
delicate  layer  of  cellular  mem- 
brane; outside  this  is  a  fibrous 
membrane,  lying  in  folds  which 
appear  as  sinuosities  in  the 
section.  The  existence  of  thia 
membrane  shows  that  the  cavity 
is  not  simply  formed  by  the 
breaking  down  of  the  tissue. 
The  tissue  is  quite  similar  in 
structure  to  that  which  is  behind 
the  commissure  in  b.* 

The  posterior  cornu  is  not 
an  uncommon  seat  of  cavities, 
which  may  extend  through  the 
whole  length  of  the  horn.  An 
example  of  this  is  shown  in  Fig. 
188.  Haemorrhage  had  occurred 
into  the  cervical  region  of  the 
cord  (a),  which  was  enlarged 
and  distended  with  blood,  so 
that  the  precise  character  of  the 
cavity  in  this  region  was  indis- 
tinguishable;  the  blood  also 
filled    the   cavity  through    the 

*  In  connection  with  the  tumour  of 
the  Cauda  equina  in  these  two  cases, 
it  may  be  mentioned  that  the  same 
coincidence  has  been  observed  in  other 
instances.  There  was  a  tumour  in  that 
situation,  for  instance,  in  the  case  de- 
scribed by  Riesinger,  who  rightly  re- 
garding the  condition  as  congenital 
in  origin,  suggests  that  the  tumour 
may  be  connected  witli  the  fact  that 
\j  '^'  the  embryonal  cord  occupied  the  entire 

^  ~  ■         length  of  the  canal. 

Fio.  188. — Sections  of  a  spinal  cord  in  which  a  cavity  existed  throughout  its  length. 
HsBmonhage  in  the  cervical  region  was  the  immediate  cause  of  death.  The  extra- 
vasation had  there  distended  the  cord  (A),  and  had  bnrst  into  the  cavity,  which 
was  filled  with  blood.  A  smaller  cavity  existed  in  the  right  cornu,  in  the  upper 
dorsal  region  (B).  In  most  parts  the  inner  surface  was  slisrlitly  irregular,  as  if  pro- 
duced by  breaking  down  of  the  tissue,  but  near  the  position  of  the  central  canal  a 
sinuous  membrane  existed  in  places,  without,  however,  any  epithelial  covering. 


SYRINGOMYELIA.  637 

entire  lengtli  of  the  cord.  In  the  upper  dorsal  region  (b)  there  are 
two  canities,  one  in  each  posterior  cornu,  but  that  on  the  right  ceased 
a  little  lower  down,  while  the  other  extended  to  the  lumbar  region, 
as  shown  in  the  figure,  reaching  almost  to  the  surface  of  the  cord. 
The  upper  and  inner  extremity  of  this  cavity  is  in  the  position  of 
the  central  canal,  no  other  trace  of  which  exists.  There  is  some 
abnormal  tissue  in  the  neighbourhood  of  the  commissure,  and  also 
in  c,  where  irregular  tracts  pass  transversely  from  the  median  septum 
into  the  fore-part  of  the  posterior  columns.  The  presence  of  this 
tissue,  and  the  relation  to  the  central  canal,  suggest  a  congenital 
origin.  During  the  reduction  in  size  of  the  canal,  in  the  development 
of  the  cord,  the  cavity  presents  at  one  period  short  lateral  processes, 
and  we  can  therefore  understand  how  a  still  earlier  arrest  of  develop- 
ment may  lead  to  the  persistence  of  a  lateral  process  on  one  side  or 
both,  sui'rounded  by  residual  embryonal  tissue,  and  that  the  cavity 
should  afterwards  extend  in  this  tissue  or  in  the  grey  matter  by  a 
process  of  growth  and  disintegration,  such  as  probably  had  occurred  in 
Fig.  188,  D.  A  comparison  of  d  and  e  suggests  that  the  two  cavities 
were  imited  in  the  middle  line  in  the  upper  part  of  the  cord,  where 
the  haemorrhage  took  place. 

In  the  conditions  we  have  hitherto  considered,  definite  limited  regions 
of  the  cord  have  been  involved  through  a  considerable  vertical  extent. 
The  changes  are  sometimes,  however,  much  more  diffuse  in  their  dis- 
tribution, as  is  shown  by  the  remarkable  lesions  represented  in  Fig. 
189.  In  this  cord,  which  is  that  of  an  adult,  we  have  a  combination 
of  the  same  two  conditions  of  abnormal  tissue  and  cavities.  Scattered 
through  the  nerve- sub  stance  are  tracts  of  a  peculiar  tissue,  represented 
by  the  darlver  shading  of  the  figure,  and  in  many  regions  thus  affected 
there  are  cavities,  for  the  most  part  fissure-like,  as  if  the  cord  had  been 
split  here  and  there.  At  first  sight  the  appearance  suggests  an  active 
and  recent  morbid  process  ;  nevertheless  I  think  that  the  congenital 
origin  of  the  condition  is  beyond  doubt.  It  will  be  observed  that  the 
morbid  changes,  as  in  the  otter  forms  of  syringomyelia,  are  chiefly  m 
the  posterior  half  of  the  cord ;  small  areas  of  disease  in  the  anterior 
columns,  in  the  sections  b  and  f,  are  the  only  exceptions.  The  minute 
structure  of  the  abnormal  tissue  was  exactly  like  that  of  the  normal 
gelatinous  layer  beneath  the  pia  mater.  Besides  the  conspicuous  areas 
figured,  it  could  be  traced  here  and  there,  in  small  tracts,  through  the 
whole  of  the  posterior  columns  and  the  hinder  parts  of  the  lateral 
columns,  and  also  in  the  posterior  horns,  in  the  grey  matter  of  which 
it  could  be  readily  distinguished.  The  relation  of  the  cavities  to  this 
substance  is  distinct ;  where  they  extend  beyond  it,  this  is  apparently 
the  result  of  the  distension  of  the  cavities  and  an  actual  fissuring  of 
the  cord,  along  the  lines  of  connective  tissue,  by  the  pressure  of  the 
contained  liquid.  The  medial  cavity  in  c  is  doubtless  due  to  defective 
closure  of  the  original  fissure  between  the  posterior  columns.  In  the 
lumbar  region  the  nerve-fibres  are  absent  from  the  front  of  the  posterior 


638  KPINAL    COKD. 

columns,  where  a  coarse  network  of  this   residual  tissue  encloses 
empty  spaces. 

The  damage  to  the  grey  matter  may  entail  alterations  in  the  related 
nerves,  and  even,  in  cases  of  long  duration,  in  those  connected  with 
the  posterior  roots.  In  the  peripheral  nerve-fibres  of  the  skin  and 
muscles,  changes  of  the  ordinary  chronic  degenerative  character  were 
found  by  Dejerine  in  cases  in  which  sensation  was  altered  or  the 
muscles  wasted. 

Thus  these  various  forms  of  syringomyelia,  in  spite  of  their  varia- 
tions in  seat  and  form  and  general  aspect,  when  closely  studied,  seem 
all  to  be  connected  with  gliomatous  tissue ;  and  that  the  morbid  state 
takes  its  origin  in  abnormal  conditions  during  the  development  of  the 
cord  is  practically  certain  from  their  seat  and  features.  This  lesson  is 
clearly  taught  by  the  facts  shown  by  the  cases  here  illustrated.  It  is 
probable,  therefore,  that  this  conclusion  is  also  true  of  cavities  of 
similar  character  and  position  in  which  no  gliomatous  tissue  can  be 
recognised ;  and  most  of  the  hypotheses  regarding  the  origin  of  such 
cavities  from  processes  of  simple  myelitis  and  the  like  have  no  suffi- 
cient foundation.  But  we  are  still  ignorant  of  the  mechanism  by 
which  the  defect  in  the  process  of  development  is  brought  about.  It 
has  been  extensively  ascribed  to  an  inflammation  of  the  embryonal 
neuroglia  at  an  early  stage  of  development,  but  of  this  there  is  no 
real  evidence.  It  is,  moreover,  no  explanation  to  assume  a  primary 
intra-uterine  hyperplasia  of  the  embryonal  tissue.  It  is,  indeed, 
possible  that  more  than  one  morbid  process  during  the  early  stage 
of  development  may  cause  the  condition,  and  that  closure  of  the 
canal  below  may  have  consequences  during  development  which  it  has 
not  at  a  later  period.  But  it  is  important  to  recognise  the  fact  that 
there  is  a  gradation  between  the  chief  varieties,  and  that  some  are 
associated  with  developmental  defects  ia  the  brain,  to  which  they  can 
only  be  related  as  the  common  expression  of  a  defect  in  the  develop- 
mental tendency  of  the  germinal  tissue ;  and  what  must  be  true  of 
some  forms  is  probably  true  of  most.  This  may  seem  only  to  push 
the  difficulty  into  the  region  of  the  inexplicable,  but  it  is  only  placing 
the  origin  of  the  malady  where  we  are  compelled  to  refer  the  causa- 
tion of  so  many  congenital  diseases,  which,  after  aU,  bear  but  a  small 
proportion  to  the  physiological  characteristics  that  spring  from  the 
same  source.  From  these  considerations  it  is  probable  that  cases  of 
syringomyelia  are  divisible  into  two  classes :  (1)  those  in  which  there 
is  a  congenital  anomaly  which  may  afterwards  take  on  a  process  of 
overgrowth  as  distinguished  from  development,  characterised  clinically 
by  very  gradual  onset  of  symptoms  lasting,  it  may  be,  throughout 
several  years ;  (2)  those  in  which  there  is  actual  central  new  growth, 
frequently  with  cavity  formation  ;  and  it  may  be  associated  with  new 
growths  elsewhere  —  glioma,  sarcoma,  syphiloma,  —  in  which  the 
course  is  a  much  more  rapidly  fatal  one. 

The  symptoms  of  the  disease  have  followed  some  illness,  mental 


SYEINGOMYELIA, 


689 


bTiocTs:  or  anxiety,  and  traumatic  influ- 
ences, but  it  is  probable  that  in  no 
case  bave  these  done  more  than  excite 
the  manifestations  of  pre-existing  dis- 
ease, or  induce  local  changes  in  the 
morbid  state.  The  possible  influence 
of  injury  has  certainly  been  over- 
estimated, since  in  some  of  the  cases 
in  which  it  seemed  effective  the  malady 
was  unquestionably  congenital,  and  the 
traumatic  influence  only  terminated  its 
latent  stage.* 

Syimptoms. — The  manifestations  of 
syringomyelia  depend  on  the  secon- 
dary processes  of  distension  and  growth, 
and  the  damage  thus  produced  to  the 
functional     elements     of    the    cord.f 

*  Among  the  chief  papers  in  which  the  sub- 
ject will  be  found  discussed  are  those  of  Scliultze 
(see  below) ;  Hellich,  *  Arch.  Boheme  d.  Med.,* 
1890,  iii.  No.  4;  Kronthal,  '  Neur.  Centralb.,' 
1888,  p.  333  J  Oppenheim,  '  Charite  Annalen/ 
1886,  p.  409;  Hoffmann  and  Schlesinger,  loc 
cit. 

t  They  have  been  fully  described  by  Schultze, 
•Zeitschrift  f.  klin.  Med.,*  1888,  p.  525;  and 
*  Verb.  Cong.  Inn.  Med.,'  188o ;  and  by  Allen 
Starr,  'Am.  Journ.  Med.  Science,'  1888,  vol.  xcv. 


^. 


\ 


) 


/ 


V 


Fig-.  189. — Cavities  in  the  spinal  cord.  A,  mid- 
cervical,  presenting  merely  some  increase  of 
Connective  tissue  in  the  left  lateral  column. 
In  B  there  are  several  cavities  having  the 
aspect  of  distended  fissures,  the  largest  in  the 
middle  line  of  the  posterior  columns  and  the 
right  posterior  cornu;  smaller  ones  in  the 
lateral  columns.  Adjacent  to  each  is  a  quan- 
tity of  dense  tissue,  which  had  a  peculiar  and 
uniform  structure.  It  consisted  of  a  very 
fine  network  of  fibres  with  minute  nuclear 
bodies  in  the  interstices.  No  larger  cells 
were  observed.  Throughout  the  posterior  and 
lateral  columns  this  tissue  could  be  seen,  here 
and  there,  thickening  the  neuroglia,  where 
no  conspicuous  masses  were  formed.  It  could 
also  be  traced  in  the  posterior  cornu,  and  was 
readily  distinguished  from  the  spongy  sub- 
stance by  its  slighter  staining.  Tlie  distri- 
liution  of  this  tissue  is  shown  in  the  other 
sections  by  the  darker  shading.  In  C  the 
fissure  in  the  middle  line  is  narrow.  In  E 
and  P  the  anterior  part  of  the  posterior 
columns  contained  a  coarse  network  of  this 
tissue,  and  no  nerve-fibres.  The  central 
can^l  w;is  obliterated,  but  the  group  of  nuclei  that  marked  its  position  eoold  bt 
traced  tiiroughouc  the  cord. 


640  SPINAL   CORD. 

Hence  the  state  is  sometimes  discovered  after  death,  when  it  has 
given  rise  to  no  symptoms,  and  its  existence  has  not  been  suspected 
during  life.  In  young  children,  indeed,  it  appears  never  to  cause  any 
definite  symptoms,  while  the  disturbance  of  function  is  variable  in 
occurrence  and  degree — much  more  so  than  in  character.  The  central 
position  of  the  disease  entails  considerable  uniformity  in  the  distri- 
bution of  its  local  consequences,  and  hence  also  in  the  derangement  of 
function  that  results  from  simple  increase  in  the  lesion  either  by  dis- 
tension or  growth.  Hence  the  symptoms  ai*e  often  such  that  they 
have  considerable  diagnostic  importance,  although  in  a  minority  of 
cases  they  are  less  uniform  in  character,  probably  because  the  disease 
is  unsymmetrical,  or  irregular  in  position  or  extension,  whether  the 
symptoms  are  due  to  the  distension  of  a  cavity,  or  to  this  combined 
with  adjacent  growth  of  tissue.  It  then  often  causes  symptoms  indis- 
tinguishable from  those  of  a  tumoiir  of  neoplastic  nature.  Moreover 
the  precise  level  at  which  the  chief  morbid  process  takes  place  leads 
to  a  corresponding  variation  in  the  position,  and  therefore  in  the 
general  aspect,  of  the  symptoms  it  produces.  The  fact  that  the  lesion 
is  almost  always  greater  at  the  cervical  region  causes  the  arms  to  be 
the  most  common  seat  of  its  manifestations,  while  the  occasional  inva- 
sion of  the  fourth  ventricle  by  the  associated  growth,  and  the  occur- 
rence of  coincident  secondary  changes  in  this  part,  explain  the  involve- 
ment of  some  of  the  cranial  nerves  that  has  been  met  with  in  a  few 
instances. 

The  symptoms  are  usually  of  slow  development,  increasing  gradually 
in  the  course  of  years.  Their  main  features  are  twofold,  of  which 
one  is  clearly  and  certainly  intelligible,  while  the  explanation  of  the 
other  is  a  matter  of  hypothesis,  although  also  not  difficult  to  under- 
stand, at  least  in  part,  if  the  opinions  expressed  in  the  chapter  on  the 
functions  of  the  cord  are  correct.  The  two  leading  features  are  loss 
of  sensibility,  chiefly  to  pain  and  temperature,  and  muscular  atrophy, 
resembling  in  characters  that  met  with  in  the  "  progressive  "  spinal 
form.  Of  the  two,  the  sensory  loss  is  the  earlier  and  more  constant. 
The  muscular  atrophy  is  a  necessary  consequence  of  the  damage  to 
the  anterior  cornua  from  the  compression  they  endure,  when  and 
where  the  central  cavity  or  adjacent  growth  attains  such  a  size  as 
to  damage  them  seriously,  and  they  may  be  reduced  to  a  mere  zone 
between  the  cavity  and  the  outer  ring  of  white  substance.  The  de- 
struction of  the  motor  nerve-cells  involves  the  degeneration  of  the 
nerves  and  the  wasting  of  the  muscles.  The  loss  of  sensation  is 
intelligible  if  the  path  of  painful  impressions  is  by  the  posterior 
comiaissure.*  The  enlargement  of  the  central  canal  and  disease  in 
its  vicinity  can  hardly  fail  to  damage  early  and  much  the  fibres  that 
cross  at  the  level  of  the  chief  lesion,  and  constitute  the  path  from 
posterior  roots  not  very  far  below.     The  course  of  thermal  sensations 

•  See  p.  224  as  to  a  tract  in  the  grey  commissure  described  by  Ciagliuski  ('Neur. 
Ceut./  1896). 


STEINGOMYBLIA.  641 

is  Btfll  qmte  tmTmown ;  the  only  fact  we  have — the  frequency  with 
which  such  sensibility  and  that  to  pain  are  involved  together — points 
to  contiguity  of  path,  and  prevents  surprise  at  the  special  affection  of 
this  form  of  sensibility  in  syringomyelia.  Tactile  sensations  are  lost 
only  in  rare  cases ;  our  ignorance  of  their  path  prevents  us  from  ex- 
plaining the  fact,  but  we  know,  from  other  facts  of  disease,  that  it  is 
distinct  from  that  for  pain.  Moreover,  the  occasional  localisation  of 
the  morbid  process  in  the  posterior  columns,  and  even  cornua,  may 
explain  the  involvement  of  all  forms  of  sensibility,  and  also  its  occa- 
sional restriction  in  area,  since  it  may  be  due  to  direct  interference 
with  the  posterior  root-fibres  after  they  have  entered  the  cord. 

In  many  cases  the  affection  of  sensation  of  pain  and  temperature  is 
equal ;  in  others  the  latter  is  lost  to  a  greater  degree  and  extent  than 
the  former.  Its  loss  is  often  the  earliest  symptom,  as  the  scars  of 
burns  may  show.  The  loss  to  heat  and  cold  may  not  correspond. 
Thermal  or  painful  hypersesthesia  has  preceded  the  loss,  as  with  other 
forms  of  sensation  when  destroyed  by  processes  that  slowly  damage 
the  fibres,  and  irritate  before  they  interrupt.  Perversion  of  sensation 
has  been  noted,  heat  being  felt  as  cold,  and  vice  versa.  Spontaneous 
sensations  are  common,  doubtless  from  the  irritation  of  the  fibres 
in  the  early  stage,  or  of  their  upper  portions  when  they  are  inter- 
rupted— feelings  of  heat  or  cold,  and  in  some  cases  pains,  various  in 
character,  sometimes  paroxysmal  like  neuralgia,  or  sharp  and  brief 
like  those  of  tabes,  and  occasionally  referred  to  the  joints,  or  felt 
chiefly  in  some  part  adjacent  to  the  seat  of  the  other  symptoms. 
Pain  in  the  spine  may  be  complained  of,  and  is,  perhaps,  a  direct 
effect  of  the  disease.  Cramps  also  and  shooting  pains  sometimes  occur. 
Often  the  malady  causes  no  suffering.  The  loss  of  sensation  is 
usually  sharply  limited,  and  the  arms  or  upper  half  of  the  trunk  are 
its  common  seat.  It  is  usually  found  in  the  same  part  as  the  mus- 
cular wasting,  but  is  more  extensive,  especially  below  the  seat  of 
atrophy.  Thus,  when  the  muscles  of  the  shoulders  and  upper  arms 
only  are  wasted,  the  sensory  loss  may  be  in  the  forearms  and  hands. 
This  we  should  expect  from  the  fact  that  the  crossing  of  the  sensory 
path  is  some  distance  above  its  entrance  into  the  cord,  while  the  motor 
roots  arise  from  nerve-cells  mostly  near  their  level  of  exit.  Exceptions 
may  be  due  to  the  affection  of  the  root-fibres,  as  explained  above. 
Further,  the  fact  that  the  distension  and  damage  always  extend 
through  a  considerable  vertical  area  of  the  cord  makes  the  relation 
Vf  the  two  sets  of  symptoms  more  extensive  and  less  regular  than  if 
the  disease  were  limited  to  a  small  region. 

The  "  muscular  sense  "  is  said  to  have  been  generally  normal  when 
tested.  Occasionally  there  has  been  unsteadiness  on  standing  with 
the  eyes  closed. 

The  motor  symptoms  usually  come  on  after  the  sensory  loss,  long 
after  in  slight  cases,  and  consist  in  muscular  weakness  and  wasting 
in  the  upper  limbs ;  while,  if  the  legs  suffer,  it  is  generally  from  simple 
VOL.  I.  41 


642  SPINAL    CORD. 

spastic  paralysis,  such  as  would  result  from,  and  is  no  doubt  due  to, 
compression  of  tlie  pyramidal  tracts  by  the  disease  in  the  upper  part 
of  the  cord.  It  is  rarely  complete.  The  unsteadiness  of  move- 
ment above  mentioned  we  may  connect  with  the  tendency  of  the 
lesion  to  involve  the  posterior  columns,  and  so  probably  to  inter- 
fere with  the  path  from  the  muscles  to  the  cerebellum.  Earely  there 
is  atrophy  of  the  legs  similar  to  that  of  the  arms,  and  in  such  cases 
the  disease  extends  into  the  lumbar  enlargement.  Eeflex  action  in 
the  legs  varies  according  to  their  state ;  it  may  be  normal  or  increased  : 
the  knee-jerks  are  often  augmented,  with  rectus-  and  foot-clonus,  or, 
in  the  cases  last  mentioned,  myotatic  irritability  may  be  lost — very 
rarely  on  one  side  only.  A  curious  tremor  of  the  limbs  has  been 
noted  in  some  cases. 

The  muscular  atrophy  in  the  arms  begins  in  and.  chiefly  afEects 
certain  parts,  according  to  the  position  of  the  greatest  damage  to  the 
grey  matter  of  the  cord.  It  may  be  in  the  hand  and  region  of  the 
alnar  nerve,  or  in  the  muscles  supplied  by  the  musculo-sjjiral  and 
radial  nerves,  or  in  the  shoulder  and  upper  arm  muscles.*  It  is  a 
slow  wasting,  with  gradual  lowering  of  the  electrical  irritability,  as  in 
progressive  muscular  atrophy,  although,  as  in  that  disease,  some 
groups  of  muscles  may  present  indications  of  the  reaction  of  degene- 
ration in  the  early  stage  of  their  affection.  Fibrillation  is  common. 
As  the  wasting  and  weakness  increase  in  the  part  first  affected,  they 
spread  to  others,  to  the  rest  of  the  limb,  and  to  the  adjacent  parts  of 
the  trunk.  Weakness  of  the  trunk  muscles  often  leads  to  lateral 
curvature  of  the  spine ;  and  the  convexity,  owing  to  posture,  is  usually 
to  the  left.  The  occurrence  of  similar  symptoms  in  the  legs  is  ex- 
tremely rare.  Both  sides  usually  suffer  together ;  a  unilateral  affec- 
tion has  indeed  been  observed,  but  is  altogether  exceptional.  The 
sphincters  may  escape  or  be  involved. 

Trophic  disturbances  in  the  extremities  affected  with  sensory  loss 
are  not  uncommon.  The  skin  may  become  thin  and  glossy,  or  thick 
and  homy.  Acute  changes  may  occur — eczema,  herpes,  or  bullae ; 
whitlows;  deep,  obstinate  ulceration,  and  even  gangrene.  More 
frequent,  however,  is  simple  vaso-motor  disturbance,  coldness  and 
lividity  of  the  extremities,  or  redness  with  swelling  and  heat,  and 
sometimes  a  tough  oedema,  which  may  be  local  like  a  tumour,  and 
afterwards  pass  away.  The  nails  share  the  trophic  changes  in  the 
hands,  becoming  grooved,  fissured,  and  cracked ;  they  may  even  drop 
off.  The  secretion  of  sweat  is  lessened  in  the  part,  as  was  well  marked 
in  one  unilateral  case,t  or  it  naay  be  increased.     The  action  of  pilo- 

•  Blocq  thinks  that  spastic  paraplegia  is  chiefly  met  with  in  the  CHses  in  which 
the  atrophy  begins  in  the  region  of  the  ulnar  nerve,  and  tabetic  symptoms  in  the 
legs  when  it  begins  in  the  radial  area  ;  but  the  data  for  the  generalisation  need  to  be 
Tnore  extensive. 

t  Allen  Starr,  'Am.  Journ.  Med.  Sc.,'  May,  1888;  Rumpf ,  •  Neur.  Cent.,'  1889, 
p.  257. 


SYiilNGOMYELIA.  643 

carpine  lias  been  fotincl  to  be  delayed,  altbotigb  ultimately  increased 
(Gri-asset).  The  bones  may  become  thickened  or  brittle,  and  joint 
changes  (Charcot's  joint),  like  those  of  tabes,  sometimes  occTir.* 

It  is  not  common  for  the  symptoms  to  extend  into  the  region  of  the 
cranial  nerves,  although  in  occasional  cases  various  disturbances  of 
function  have  occurred,  presumably  due  to  the  extension  of  the  changes 
into  the  medulla  and  pons,  and  chiefly  met  with  vfhen  there  is  upward 
extension  of  the  associated  growth,  already  mentioned.  Thus  there 
have  been  paralysis  of  one  vocal  cord,  or  of  both,  of  the  tongue,  the 
palate,  and  of  the  face,  difficulty  in  swallowing,  disorder  of  respiration 
and  of  the  heart's  action.  Taste  and  smell  may  be  affected,  and  there 
may  be  sensory  impairment  in  the  face.  Certain  disorders  may 
occur  in  the  eyes,  probably  due  to  disease  of  the  path  of  the  sympa- 
thetic in  the  cord,  or  to  that  of  the  fourth  ventricle.  Thus  the 
pupils  are  often  imequal,  the  smaller  being  on  the  side  of  the 
more  severe  cord  symptoms,  on  which  the  fibres  for  the  radiating 
muscle  of  the  iris  are  likely  to  be  damaged.  Narrowing  of  the 
palpebral  fissure  and  slight  ptosis  have  been  also  noted.  Nystagmus 
is  not  rare.f 

The  course  of  syringomyelia  is,  as  a  rule,  slow,  and  measured  by 
years,  although  an  apparent  exception  is  presented  by  the  cases  in  which 
it  is  associated  with  spreading  overgrowth  of  the  adjacent  gliomatous 
tissue,  or  in  which  acute  destructive  processes  occur  in  this  and 
spread  to  its  vicinity.  In  such  cases  a  considerable  development  of 
the  disease  may  be  attained  before  symptoms  are  produced,  and  these 
may  rapidly  reach  a  considerable  degree,  and  cause  death  in  a  few 
months  or  even  less.  In  most  cases  death  is  the  result  of  exhaustion, 
of  the  impairment  of  the  functions  of  the  medulla,  or,  more  com- 
monly, of  some  complication — bedsores,  cystitis,  and  the  like. 

Diagnosis. — The  resemblance  of  the  disease  to  more  common 
maladies  led  to  its  confusion  with  them  during  life,  until  the 
careful  observations  of  the  last  few  years  have  established  features  by 
which  its  existence  can  often  be  at  least  surmised.  The  most  im- 
portant characteristic  is  the  combination  of  muscular  atrophy  with 
gtUl  earlier  sensory  loss,  involving  temperature  and  pain  far  more 
than  tactile  sensibility.  But  the  occasional  implication  of  all  forms 
of  sensation  must  be  kept  in  mind,  and  also  the  fact  that  in  slight 
and  slow  or  anomalous  cases  the  sensory  symptoms  may  long  predomi- 
nate, and  for  a  time  exist  alone.  The  position  of  these  symptoms 
(in  the  arms),  and  their  frequent  combination  with  spastic  palsy  in 
the  legs,  is  a  further  characteristic — not,  however,  unequivocal. 

Cervical    pachymeningitis     causes     symptoms    which,   in  general 

*  Nissin,  *Arch.  f.  klin.  Chir.,'  Bd.  xlr;  *  Neur.  Centralb.,'  1893,  p.  100; 
Sokoloff,  *  Deutsch.  Ztschr.  f.  Chir.,'  Bd.  xxxiv;  *Neur.  Centralb.,'  1893,  p.  101; 
Galloway, '  Lancet,'  1891,  vol.  i. 

t  Contraction  of  the  fields  of  vision,  especially  for  colours,  has  h'en  observed,  but 
■  not  easy  to  explain  (Dejerine  and  Turland), 


644  SPINAL    COKD. 

character  and  in  distribution,  alike  in  tlie  arms  and  fhe  lerjs, 
very  closely  resemble  those  of  syringomyelia  ;  but  the  affectioii 
runs  a  more  rapid  course,  the  anaesthesia  involves  all  forms  of 
sensibility,  and  has  not  a  greater  but  a  less  estent  than  the  muscular 
■wasting ;  the  two  correspond  more  closely  in  their  seat ;  pain  is  a 
far  more  prominent  symptom,  and  the  reaction  of  degeneration  is 
common  in  the  wasting  muscles.  Tumours  of  the  spinal  cord  only 
cause  symptoms  of  like  character,  and  especially  of  similar  symmetry, 
when  they  are  central  in  situation,  and  the  diagnosis  may  then  be 
very  difficult  and  even  impossible.  But  the  resemblance  is  often 
more  than  superficial,  for  such  growths  generally  arise  from  residual 
gliomatous  tissue,  and  are  such  as  are  frequently  associated  with 
syringomyelia.  It  is  chiefly  from  the  more  rapid  development  of 
symptoms,  and  their  more  speedy  extension,  that  such  a  growth  can 
be  suspected;  and  only  the  presence  of  slighter  chronic  preceding 
symptoms  of  the  character  above  described  would  justify  the  expecta- 
tion that  a  cavity  is  associated  with  the  growth. 

Progressive  muscular  atrophy  is  sufficiently  distinguished  by  the 
absence  of  impairment  of  sensation,  and  in  peripheral  neuritis  the 
pains,  mode  of  onset,  and  degenerative  reaction  in  the  muscles  should 
preclude  any  danger  of  mistake.  In  "  Morvan's  disease,"  to  be  pre- 
sently described,  the  symptoms  may  bear  a  close  resemblance  to  those 
of  syringomyelia,  and  it  is  probable  that  they  are  due  to  a  similar 
condition  of  the  spinal  cord,  so  that  it  is  to  be  recognised  merely  as  a 
variety  of  syringomyelia.  As  a  rule,  however,  the  early  loss  of  tactile 
sensibility,  and  the  greater  prominence  of  trophic  disturbance  in  the 
tissues,  especially  the  painless  whitlows,  enable  the  diagnosis  to  be 
made.  There  is  only  one  acute  affection  of  the  cord  that  enters  into 
the  diagnostic  problem,  and  that  is  spinal  haemorrhage.  It  has  been 
mentioned  in  the  account  of  this  condition  that  the  haemorrhage  is 
sometimes  into  a  pre-existing  cavity — as  in  the  case  shown  in 
Fig.  188.  The  presence  of  a  cavity  into  which  the  blood  has  been 
effused  may  be  suspected  if  the  haemorrhage  has  been  preceded  by 
any  symptoms  suggestive  of  syringomyelia,  inquiry  for  which  should 
always  be  made ;  or  if  a  rapid  extension  occurs  through  a  consider- 
able vertical  extent  of  the  cord,  distinctly  abolishing  its  central 
mnctions. 

The  malady,  however,  varies  much  in  seat  and  characters,  as  is 
evident  from  even  a  cursory  glance  at  the  illustrations  given  above; 
the  symptoms,  of  necessity,  vary  with  the  lesion.  The  diagnosis  may 
be  difficult  and  even  impossible  in  such  cases,  especially  in  the  early 
stages  and  slighter  forms,  when  irritation  and  pain  predominate,  and 
even  neuralgia  may  be  simulated.  It  may  be  necessary  to  wait  for 
time  to  show  the  course  of  the  symptoms.  Their  progressive  increase, 
in  spite  of  variations,  should  always  be  taken  into  account;  it  fre- 
quently is  the  first  feature  that  suggests  the  nature  of  such  a  disease, 
contrasting,  as  it  does,  with  the  tendency  of  inflammatory  affections 


ANALGIC    PANAEITIUM,  645 

to  arrest  and  improvement.  A  peculiar  difficulty  is  presented  by  the 
cases,  not  few,  in  which  the  first  symptoms  follow  some  influence — 
an  illness,  a  blow,  or  even  mental  disturbance — that  seems  to  be  their 
cause,  and  suggests  disease  of  very  diif erent  nature.  Often  then,  also, 
it  is  only  by  waiting  and  watching  that  a  right  opinion  can  be  formed. 
When  bulbar  symptoms  come  on  rapidly,  the  nature  of  the  case  can 
only  be  inferred  from  previous  spinal  symptoms. 

The  Prognosis  and  Treatment  of  syringomyelia  are  subjects  on 
which,  lanfortunately,  its  nature  permits  little  to  be  said.  If  its 
existence  can  be  recognised  the  malady  is  generally  advanced,  and  its 
observed  rate  of  progress  is  the  only  ground  on  which  a  forecast  can 
be  based.  When  the  central  functions  of  the  cord  are  considerably 
impaired,  and  the  symptoms  are  steadily  increasing,  it  is  rare  for  life 
to  be  prolonged  for  more  than  one  or  two  years,  and  the  subacute 
onset  of  considerable  paralysis  may  herald  a  course  that  leads  to 
death  in  a  few  months.  On  the  other  hand,  a  stationary  condition 
may  last  for  many  years.  Treatment  can  do  nothing  for  the  morbid 
state,  nor  is  it  likely  that  the  progress  of  spinal  surgery  can  afford  the 
means  of  relief.*  The  severity  of  some  of  the  individual  symptoms 
may,  however,  be  lessened  by  appropriate  measures ;  especially  trophic 
changes  may  be  diminished  by  care  and  early  treatment,  bedsores 
may  be  prevented,  cystitis  guarded  against,  and  if  any  acute  para- 
lysis occurs,  the  possibility  of  some  recovery  may  suggest  the  main- 
tenance of  the  irritability  of  the  muscles  by  electricity,  which  cannot 
be  expected  to  influence  the  slower  atroj^hy.  Fain  may  need  the 
customary  sedatives  ;  but  early  pain,  especially  with  the  hypersesthesia 
that  depends  on  the  intensification  of  the  nerve-impulses  at  the  irritated 
part,  may  be  much  lessened  by  the  administration  of  cocaine, 
used  as  in  tabes.  Sudden  pain  in  the  spine  may  indicate  commencing 
haemorrhage,  and  should  lead  to  the  adoption  of  the  posture  and  other 
measures  appropriate  to  that  grave  aiSection. 


AkaIiQIO  PANABirnrM;  Moevan's  Biseabb. 

By  the  term  Morvan's  Disease  is  generally  known  an  affection  that 
was  called  Panaritium  Analgicum,  or  "  Painless  Whitlows,"  by  the 
physician  of  Brittany,  Morvan,  who,  in  1883,  first  described  it.f 
Observations  on  it  are  stiU.  few,:j:  but,  judging  from  two  fatal  cases,  §  it 

•  In  one  case  under  my  care  the  spinal  canal  was  opened  and  the  cavity  drained, 
but  without  improvement. 

t  'Gaz.  Hebd.,'  1883,  Nos.  35—44. 

J  The  chief  writings  are  those  of  Louazel,  *  La  Maladie  de  Morvan,  Paris,  1890; 
Jeffrey  and  Achard,  'Arch,  de  Med.  exp./  1890 j  Charcot,  'Prog.  Med.,'  1890, 
No8.  11  and  12 ;  Hackel, '  Miinch.  med.  Wochenschr.,'  1889. 

§  Gombault,  quoted  by  Charcot;  Jeffrey  and  Achard,  'Arch,  de  Med.  exp.,'  1891. 


646  SPINAL  COED. 

appears  to  consist  in  a  combination  of  a  condition  of  syringomyelia, 
or  of  the  associated  gliomatosis,  with  a  peripheral  neuritis  in  the  extre- 
mities. The  symptoms  make  the  constancy  of  the  neuritis  probable ; 
we  must  wait  to  learn  how  far  the  morbid  state  of  the  cord  is  constant, 
and  whether  the  changes  in  the  nerves  take  origin  in  developmental 
conditions.  It  is  quite  possible  that  they  may  share  the  condition  of 
arrested  development,  entailing  a  liability  to  later  morbid  changes, 
especially  in  view  of  the  analogous  conditions  observed  in  the  case 
mentioned  on  p.  629.*  At  the  same  time  it  is  possible  that  the 
symptoms  of  this  affection  may  be  due  to  local  neuritis  of  varied 
nature,  and  we  must  be  cautious  in  inferring  from  them  that  the 
pathological  state  is  the  same  in  origin  in  all  cases.  In  some  instances 
the  malady  has  been  apparently  set  up  by  an  injury.  The  affection 
has  been  met  with  in  both  sexes,  but  more  frequently  in  males,  and 
has  generally  commenced  during  the  first  half  of  adult  life,  betweea 
twenty  and  forty.  When  local  injury  has  preceded  the  symptoms  it 
has  sometimes  been  at  a  considerable  interval  (Hackel),  and  it  has 
been  unilateral  whenthe  latter  have  been  bilateral,  so  that  its  relation 
to  them  is  uncertain. 

The  characteristic  symptoms  are  in  the  upper  extremities ;  only  in 
rare  cases  do  the  feet  suffer  at  a  late  period.  Neuralgic  pains  may 
occur  first,  but  the  definite  symptoms  consist  of  weakness  and  muscular 
wasting  in  the  hands  and  forearms,  loss  of  all  forms  of  sensation,  and 
especially  a  peculiar  trophic  change — whitlows  on  the  fingers,  with 
recurring  deep  ulcerations  in  various  parts  of  the  digits ;  those  near 
the  extremity  of  the  fingers  may  only  heal  with  the  loss  of  some  of  the 
terminal  phalanges.  The  loss  of  sensibility  involves  all  forms,  and 
usually  precedes  the  occurrence  of  the  whitlows,  so  that  these  are 
painless,  a  peculiarity  which  constitutes  the  most  salient  feature  of  the 
disease,  and  suggested,  in  the  first  instance,  its  special  character. 
Occasionally  the  trophic  disturbance  precedes  the  anaesthesia,  and  the 
whitlows  and  ulcers  are  then  painful.  The  latter  are  deep,  a;nd  often 
resemble  the  perforating  ulcers  of  tabes ;  cracks  in  the  skin  accompany 
them,  and  the  nails  shrivel  and  split.  Vaso-motor  derangements, 
lividity  and  pallor,  often  precede  and  accompany  the  disturbance  of 
nutrition.  The  progress  of  the  affection  is  very  slow,  and  extends  over 
many  years ;  one  hand  is  usually  affected  some  time  before  the  other. 
The  electrical  irritability  of  the  nerves  of  the  part  has  been  found 
normal  in  the  early  stage,  and  slowly  vanishing  as  the  malady  pro- 
gressed. Although  the  trophic  changes  are  limited,  as  a  rule,  to  the 
hands,  and  the  muscular  wasting  does  not  extend  above  the  forearms, 
the  loss  of  sensation  is  occasionally  more  extensive,  involving  the 
whole  arms,  parts  of  the  trunk,  and  even  the  face.     An  affection  of 

*  In  a  case  recorded  by  Jolly  ('  Charite  Ann.,'  xvi,  1891)  there  was  also  a  coa» 
genital  anomaly  of  the  fingers,  a  web  of  skin  between  the  third  and  fourth  fingen 
of  each  hand. 


SPINA   BIFIDA.  647 

tLe  shoulder-joint  has  been  observed.*  The  feet  are  occasionally, 
though  rarely,  the  seat  of  anaesthetic  ulceration ;  the  legs  have  also 
been  found  weak,  with  excessive  knee-jerk  and  foot-clonus  (Hackel). 

In  the  autopsy  that  was  made  by  Grombault  neuritis  was  found  in 
both  arms,  and  also  a  diffuse  overgrowth  of  connective  tissue  in  the 
posterior  part  of  the  grey  matter  of  the  cord  and  the  posterior  columns. 
This  growth  involved  also  the  coats  of  the  vessels,  which  were  in  places 
so  thickened  as  to  obliterate  their  cavity.  Neuritis  has  also  been 
invariably  found  in  the  extremities  of  the  fingers  when  these  have 
dropped  off. 

The  features  of  the  malady  are  so  peculiar  that,  if  they  are  known, 
the  nature  of  a  case  cannot  well  be  mistaken.  Anaesthetic  leprosy 
presents  the  closest  resemblance,  but  in  this  the  peculiar  ulcerations 
are  absent,  and  pigmentary  alterations  occur  in  the  skin ;  while  in 
Raynaud's  disease  there  is  not  the  peculiar  loss  of  sensibility,  nor  an 
equal  tendency  to  whitlows,  and  vaso-motor  disturbance  is  a  more 
conspicuous  feature.  In  the  peculiar  malady  known  as  "sclero- 
dactyla  "  (a  form  of  scleroderma  that  affects  the  fingers  and  face) 
there  is  no  loss  of  sensibility  or  tendency  to  destruction  of  the  finger 
ends.  Simple  syringomyelia  is  distinguished  by  the  common  absence 
of  the  whitlows,  by  the  subordination,  in  time  and  degree,  of  the 
trophic  changes  to  the  other  symptoms,  and  by  the  common  preserva- 
tion of  tactile  sensibility.  But  the  distinction  is  probably  not  an 
absolute  one.  In  Morvan's  disease  there  is  peripheral  neuritis  as  well 
as  syringomyelia,  and  to  this  is  due  its  special  features,  and  especially 
the  early  and  intense  trophic  disturbance.f 


Spina  Bifida. 

Spina  Bifida,  or  split  spine,  depends  on  a  defect  in  the  closure  of 
the  vertebral  arches,  which  usually  leads  to  the  protrusion  of  the 
membranes  as  a  sac,  forming  an  external  tumour,  into  which  the  lower 
part  of  the  spinal  cord  often  extends,  normal  or  variously  altered  in 
conformation,  sometimes  even  reduced  to  a  neural  lining  of  part  of  the 
wall.  Rarely  there  is  no  external  prominence — a  form  known  as 
"  spina  bifida  occulta."  J  The  condition  is  most  common  in  the  lumbar 
region  of  the  spine ;  sometimes  is  present  at  more  than  one  place,  very 
rarely  in  the  whole  length  of  the  vertebral  column.  It  is  met  with 
in   about  one  child  out  of  every  thousand  born  (Chaussier).     The 

•  Morvan, '  Gaz.  Hebd,,'  1887,  No.  34. 

f  The  absence  of  any  fundamental  difference  between  this  malady  and  syringo- 
myelia has  been  pointed  out  by  Bernhardt  ('  Verb.  Vereins  inn.  Med.,'  Berlin, 
January  19tli,  1891)  and  Jolly  (loc.  cit.).  But  the  additional  element  of  neuritis 
must  not  be  ignored. 

J  The  best  accounts  of  spina  bifida  are  in  the  "  Report  of  the  Committee  of  the 
Clinical  Society"  ('Transactions,'  1885),  and  Bland  Sutton's  "Lectures  on  EvoIutioB 
in  Pathology"  ('Lancet,'  February  25th,  1888). 


648  SPINAL    CORD. 

disease  is  one  of  chiefly  surgical  interest,  and  therefore  only  an  outline 
of  the  more  important  facts  is  here  given.  The  spinal  cord,  whether 
normal  or  not,  generally  reaches  lower  than  usual  in  the  vertebral 
canal,  as  it  does  at  the  early  period  of  development,  when  the  morbid 
state  is  produced, — becoming  adherent  at  its  lower  extremity  and 
remaining  so.  In  many  cases  the  central  canal  of  the  cord  is  enlarged 
above,  and  the  lower  part  of  the  cord  may  also  be  similarly  distended. 

The  precise  condition  presents  many  variations,  which  can  be,  in  part 
at  least,  understood  by  what  is  known  of  the  process  of  development,  a 
subject  that  has  been  very  ably  discussed  by  Bland  Sutton.  The 
essential  element  in  spina  bifida,  the  defect  in  the  vertebral  arches, 
depends  on  a  deficiency  in  that  ingrowth  of  the  mesoblast  from  each 
side  which  should  enclose  the  embryonal  spinal  cord,  and  fonn  bone, 
after  the  superficial  epiblastic  layers  have  united  to  form  the  epidermis. 
A  similar  ingrowth  of  mesoblast  forms  the  corium,  muscle,  &c.  This 
apparently  takes  place  in  excess  in  spina  bifida  occulta,  and  leads  to 
a  curious  condition,  usually  present — an  excessive  growth  of  hair  over 
and  about  the  affected  part,  which  is  generally  the  lumbo-sacral 
region.  On  the  other  hand,  in  many  cases  in  which  there  is  a  tumour, 
this  superficial  mesoblastic  ingrowth  seems  to  be  deficient,  as  well  as 
that  for  the  vertebral  arches,  so  that  at  the  upper  part  of  the  tumour 
there  is  an  area  in  which  there  is  no  proper  skin,  but  a  peculiar  shiny 
membrane  destitute  of  hair.  It  is  probably  this  superficial  mesoblastic 
deficiency  that  permits  the  protrusion,  and  formation  of  an  external 
tumour. 

In  spina  bifida  occulta  the  defect  in  the  arches  can  be  felt  on 
palpation,  either  in  the  lumbar  or  sacral  region,  and  the  spinal  cord 
extends  lower  than  normal,  as  in  the  ordinary  form.  The  latter,  in 
which  there  is   an  external  tumour,  presents   three   chief  varieties. 


Fig.  190. — Spina  bifida:  sections  of  a  spinal  cord,  from  a  case  the  symptoms  and 
history  of  which  I  was  unnble  to  ascertain.  In  A,  cervical  region,  tlie  only  abnor- 
mality is  a  large  cruciform  ceniral  canal  and  an  unusually  thick  grey  commissure. 
This  continues  through  the  dorsal  region,  at  tlie  lowest  part  of  whicli  there  is  a 
great  change.  In  B,  the  canal  is  larger,  and  the  necks  of  the  posterior  horns  (and 
posterior  vesicular  columns)  are  united  by  much  commissural  tissue;  in  this,  many 
vertical  fibres  could  be  seen,  and  others  running  from  before  backwards  in  the 
middle  line.  In  C,  an  extension  of  the  canal  backwards  has  taken  place,  and  it  is 
bounded  by  a  sinuous  niembriine;  The  outer  part  of  the  grey  substance  is  atro- 
phied. In  D,  the  cavity  has  extended  into  each  posterior  horn,  almost  up  to  the 
surface  of  the  cord,  in  part  by  a  breaking  down  of  tissue,  because  the  membi-ane 
limiting  the  enlarged  canal  remains  undestroyed.  In  E,  still  at  the  junction 
of  the  dor-al  and  lumbar  regions,  the  cavity  has  receded  from  the  left  horn.  In 
P,  the  division  of  the  cord  has  taken  place,  not  in  the  direction  of  the  cavity  in 
the  horn  (which  is  filled  up),  but  in  the  middle  line,  at  or  close  to  the  median 
septum.  In  G,  a  wider  separation  of  the  posterior  columns  has  taken  place,  and  a 
cavity  has  formed  on  the  right  side,  which  extends  into  the  horn,  and  almost  cuts 
ofi"  the  posterior  column.  In  H,  the  cord  is  spread  out  and  formed  part  of  the  wall 
of  the  sac.  The  posterior  nerve-roots  (jp  r)  mark  the  position  at  which  the 
posterior  horn  comes  to  the  surface.  The  grey  matter  is  in  many  parts  atrophied 
and  translucent,  and,  in  H,  contains  many  large  vessels.  The  septal  lobulation  on 
the  surface  of  the  c^rd  is  greater  than  normal. 


SPINA    BIFIDA. 
Fig.  190. 


649 


650  SPINAL    CORD. 

according  as  the  sac  contains  only  the  spinal  membranes  (meningocele), 
the  spinal  cord  as  weU  as  the  membranes  (meningomyelocele),  or  the 
latter  distended  by  enlargement  of  the  central  cavity  (syringo- 
myelocele). These  are  the  chief  classes;  they  do  not,  indeed,  exhaust 
the  rarer  forms,  but  to  take  fidl  cognizance  of  these  would  entail  a 
very  complex  list  of  varieties.* 

Of  the  several  forms,  that  without  an  external  tumour  has  been 
least  frequently  met  with,  although  it  is  probable  that  a  knowledge  of 
the  significance  of  the  growth  of  hair  in  the  lumbar  region  would  lead 
to  the  detection  of  this  state  in  many  cases  in  which  it  is  now 
undiscovered.  Putting  this  form  aside,  simple  meningocele  and 
syringomyelocele  are  both  rare ;  the  common  form  is  meningo- 
myelocele, in  wliich  the  cord,  altered  or  intact,  extends  within  the  sac. 
The  Clinical  Society's  Committee  found  that  this  was  the  condition  in 
62  per  cent,  of  the  cases  in  which  there  was  an  external  tumour.  The 
lower  part  of  the  spinal  cord  is  generally  adherent  to  the  posterior 
wall  of  the  sac,  where  its  traction  often  causes  a  depression  on  the 
surface,  always  at  the  membranous  area,  which,  as  already  mentioned, 
is  generally  to  be  observed  in  the  upper  portion.  The  cord  may  again 
become  free,  and  extend  downwards  in  the  cavity,  or  it  may  be 
flattened,  expanded,  and  lost  in  the  wall,  of  which  its  tissue  really 
forms  an  inner  layer.  In  this  layer  there  is  no  distinction  of  grey  and 
white  substance.  In  either  case  the  lower  nerves  arise  in  the  wall,  and 
pass,  first  in  this,  and  then  forwards,  across  the  cavity,  to  their  fora- 
mina of  exit.  The  arachnoid  always  extends  into  the  sac,  and  the 
fluid  is  that  contained  within  the  subarachnoid  space;  sometimes 
there  is  an  external  opening  from  which  the  fluid  flows. 

As  a  rule,  the  central  canal  of  the  cord  is  not  continuous  with  the 
sac ;  and  it  may  be  closed,  even  in  the  condition  of  syringomyelocele,  in 
which  the  canal  is  dilated.  In  other  cases  the  canal  opens  into  the 
cavity,  sometimes  by  only  a  small  aperture,  even  when  the  lower  part  of 
the  cord  expands  into  the  wall,  and  the  nerves  course  along  the  w;all 
in  a  layer  continuous  with  the  arachnoid  and  superficial  to  the 
membrane  that  represents  the  cord -tissue  from  which  they  arise. 
As  stated,  ordinary  syringomyelia  may  exist  in  the  upper  part  of  the 
cord.  The  extension  of  the  cord  in  the  wall  of  the  sac  probably 
indicates  a  developmental  defect  similar  to  that  of  the  bony  canal ; 
in  the  lumbar  region  the  primitive  canal  has  failed  to  close,  so  that 
the  cord  is  open  posteriorly,  and  the  two  posterior  columns  may  even 
be  far  apart,  or  the  cord  may  even  be  applied,  in  the  form  of  a  thick 
or  thin  lamina,  to  the  wall  of  the  sac. 

An  instructive  although  rare  example  of  the  involvement  of  the 
cord  in  the  developmental  effect  is  shown  in  Fig.  190,  which  illus- 
trates also  the  manner  in  which  the  latter  may  involve  in  some  degree 
the  whole  cord.  In  the  cervical  region  the  grey  commissure  is 
unusually  large,  and  the  canal  is  cruciform,  a  shape  which  it  presents 
*  Several  otlier  varieties  are  euumerated  by  Lilnud  Sutton  (loc.  eit.). 


SPINA   BIFIDA.  651 

at  one  period  of  development,  and  which  is  here  persistent.  This 
condition  obtains  throughout  the  dorsal  region  until,  in  the  lower 
part,  the  tissue  behind  the  commissure  is  so  abundant  as  to  unite  the 
necks  of  the  posterior  horns  Below  this  the  canal  enlarges  into  a 
condition  of  syringomyelia,  surrounded  by  the  abnormal  tissue  which, 
as  we  have  seen,  is  commonly  associated  with  such  enlargement ;  the 
canal  is  limited,  moreover,  by  the  sinuous  membrane  that  is  so  often 
met  with  (see  p.  633).  The  cavity  extends  also  into  the  posterior 
horns,  clearly  by  a  process  of  disintegration,  since  in  d  the  membrane 
lining  the  enlarged  canal  persists,  and  marks  the  limit  of  the  canal  and 
the  extent  of  the  simple  disintegration.  The  progressive  separation  of 
the  posterior  columns  is  shown  in  f,  g,  and  h.  In  the  lowest  part 
of  the  cord,  the  development  of  the  posterior  columns  has  been  appa- 
rently hindered,  as  will  be  observed  if  q  be  compared  with  f.  In  the 
former  there  are  also  some  fissures  in  the  grey  substance,  formed 
apparently  by  disintegration.  The  grey  substance  of  the  cord  is  con- 
spicuously wasted  in  the  lumbar  region,  thin  and  translucent. 

In  rare  cases  there  has  been  no  proper  development  of  the  cord. 
It  was  represented  by  a  mere  nerve-like  string  in  a  child,  who  also  had 
hydrocephalus,  but  lived  five  weeks.  Another  child  of  the  same  parents 
had  a  similar  condition.* 

Symptoms. — If  the  cord  is  intact,  symptoms  may  be  absent.  In 
the  cases  in  which  there  is  an  external  opening,  and  a  constant  escape 
of  cerebro- spinal  fluid,  the  child  usually  dies  in  a  few  weeks.  In 
other  cases  the  defective  development  of  the  lower  part  of  the  cord, 
or  its  distension  in  syringomyelocele,  is  manifested  by  an  absence  of 
function,  amounting  to  complete  paraplegia  in  severe  cases,  which 
also  seldom  live.  Some  cases,  however,  survive,  with  paralysis  and 
wasting  of  the  legs,  and  often  absence  of  power  over  the  sphincters. 
In  slighter  forms  the  paralysis  and  wasting  have  been  partial,  either 
existing  from  the  first,  or  supervening  at  some  later  period,  probably 
from  the  occurrence  of  damage  to  the  nerve-roots  or  cord-tissue  by  in- 
creased tension,  or  by  inflammation;  of  the  latter  abundant  traces 
may  be  found  after  death,  and  various  external  influences  may  readily 
set  it  up.  Some  children,  whose  legs  have  appeared  natural,  have  never 
been  able  to  stand ;  possibly  the  defect  in  the  posterior  columns  has 
given  rise  to  a  sort  of  congenital  tabes.  Even  in  the  slighter  forms, 
including  spina  bifida  occulta,  paralysis  and  atrophy  have  come  on 
after  some  years,  and  even  after  adult  life  has  been  reached.  Indeed, 
in  the  unsuspected  forms  without  a  tumour,  there  may  be  special 
danger  of  injury  to  the  unprotected  structures,  and  damage  to  the 
nerve-roots.  The  muscular  paralysis  and  atrophy  have  been  usually 
below  the  knees,  and  the  muscles  least  prone  to  suffer  are  the  tibiales 
antici.  Hence  talipes  varus  tends  to  occur.  These  muscles  may 
alone  persist,  probably  from  the  higber  position  of  origin  of  their 
nerves  (Eemak).  Another  pecxdiarity  of  these  cases  is  the  tendency 
*  S.  Wolfe,  '  Phil.  Kep.,'  June  2nd,  1888. 


652  SPINAL  COED. 

to  perforating  ulcer  in  tlie  feet,  wMcli  lias  several  times  necessitated 
amputation.  Chronic  changes  in  the  tarsal  and  metatarsal  bonea 
have  also  been  met  with,  somevs^hat  like  those  of  tabetic  arthro- 
pathy.* The  ulcers  suggest  neuritis,  vrhich  was  found  in  one  case ; 
and,  in  this  and  others,  the  arteries  have  presented  thickening  of  the 
muscular  coat,  even  leading  to  their  closure.  These  symptoms  may 
be  greater  on,  or  confined  to,  one  side,  and  on  this  the  knee-jerk  has 
been  lessened.f  These  points  of  resemblance  to  tabes  may  also  be  due 
to  the  greater  degree  of  exposure  to  damage  of  the  posterior  roots,  as 
well  as  to  neuritis,  perhaps  descending. 

The  treatment  of  spina  bifida  is  purely  surgical.  That  of  the  con- 
sequences just  described  is  the  same  as  for  similar  states  in  other 
diseases. 


TEAUMATIO  LESION'S  OF  THE  SPINAL  CORD. 

Injuries  to  the  cord  fall  within  the  province  of  the  surgeon,  but 
some  account  of  them  is  needed  because  the  subjects  often  afterwards 
come  under  medical  observation,  and  because  the  cases  present  every 
gradation  to  the  maladies  that  have  been  described  in  the  preceding 
pages. 

Causes. — In  fractures  and  dislocations  of  the  sjDine,  the  cord  gene- 
rally suffers  compression  or  laceration,  as  already  described  in  the 
chapter  on  injuj-ies  of  the  vertebral  column.  The  cord  may  also  be 
directly  wounded  by  stabs  and  gunshot  injuries,  in  which  it  may  be 
divided  completely  or  partially.  Such  injuries  furnish  a  considerable 
proportion  of  the  cases  of  unilateral  lesion  of  the  cord.  In  gunshot 
wounds  the  cord  more  often  suffers  from  displaced  fragments  of  bone 
than  from  the  ball  itseK. 

Par  more  common  than  direct  injury  is  damage  to  the  cord  by  con- 
cussion of  the  spine,  either  local  or  general.  Gunshot  wounds  occa- 
sionally furnish  examples  of  local  concussion ;  a  bullet  may  strike  the 
spinal  column  and  lodge  in  its  vicinity,  with  the  effect  of  causing 
immediate  paraplegia,  as  complete  as  if  the  cord  were  divided,  and  yet 
it  may  be  found  that  the  spinal  column  has  not  been  injured,  and  the 
cord  is  merely  softened  at  the  spot.  Other  causes  of  concussion  are 
the  fall  of  heavy  bodies  on  the  back,  such  as  a  beam  of  timber  or  a 

*  In  one  case  Sutton  found  the  compact  tissue  very  scanty,  and  the  bones  filled 
with  semi-fluid  fat;  a  deep  ulcer  reached  a  carious  spot. 

t  Di'tails  of  cases  by  Fischer,  v.  Recklinghausen,  Brunna,  and  others,  are  quoted 
by  Bland  Suttoa  (loe.  cit.).  See  also  Delafosse,  'These  de  Paris,'  1874;  Eemak, 
'Berlin,  kl.  WochenschiiCt,'  1885, 


TKAUMATIO   LESIONS.  653 

sack  of  com,  a  blow  on  the  back  from  some  blnnt  weapon,  a  fall 
upon  the  back,  either  on  a  flat  surface  or  on  some  projecting  object. 
Less  frequently  the  cord  suffers  from  a  general  concussion  of  the 
body,  in  which  the  spinal  column  is  not  specially  involved.  The 
cervical  region  is  occasionally  damaged  in  falls  on  the  head.  Eailway 
accidents  are  frequent  causes  of  concussion  of  the  spinal  cord;  the 
back  may  be  struck  with  violence  when  the  body  is  thrown  from  one 
side  of  the  carriage  to  the  other.  Another  common  cause  is  a  fall 
•  from  a  horse  upon  the  back,  or  a  fall  downstairs,  in  which  the  spinal 
column  is  bumped  against  the  edges  of  the  steps.  A  violent  bending 
of  the  spine  may  also  injure  the  cord  directly,  especially  in  the  more 
mobile  cervical  portion,  where  the  region  of  the  fourth  and  fifth 
vertebrae  is  especially  apt  thus  to  suffer  (Thorburn).*  The  jar  of  the 
spine  caused  by  a  jump  from  a  height  may  even  be  effective.  Occa- 
sionally effects  very  similar  to  those  which  are  produced  by  a  blow  on 
the  spine  are  caused  by  a  sudden  contraction  of  the  spinal  muscles  in 
some  violent  effort,  a  "  rick  of  the  back,"  as  it  is  popularly  termed. 
It  is  possible  that,  in  such  cases,  the  primary  damage  is  sometimes  to 
the  vertebral  ligaments  and  articulations,  and  that  the  cord  suffers 
secondarily.  The  symptoms  may  only  come  on  a  few  hours  after  the 
injury  in  such  cases,  as  if  effused  blood  compressed  the  cord,  or  the 
tension  upon  it  and  slight  damage  set  up  a  graver  inflammation. 
Thus  a  soldier,  in  a  drunken  quarrel,  was  extended  by  four  comrades, 
two  pulling  on  his  feet  and  two  on  his  arms,  while  a  fifth  sat  on  his 
back.  No  immediate  effect  followed,  but  the  next  morning  there  was 
complete  paralysis  of  both  arms  and  legs,  which  very  slowly  passed 
away. 

PATHOiiOGT. — The  anatomical  lesions  in  cases  in  which  the  vertebral 
column  is  not  injured  vary  much  in  different  cases.  Haemorrhage  is 
often  found,  sometimes  outside  the  dura  mater,  sometimes  on  the 
inner  surface  of  the  membrane,  in  the  pia  mater,  in  the  substance  of 
the  cord  itseK,  very  rarely  into  the  central  canal,  f  Occasionally  the 
substance  of  the  cord  has  been  found  lacerated  when  the  vertebral 
column  has  not  been  injured.  In  many  cases  there  is  local  softenino-, 
commonly  yellow  in  tint,  sometimes  mingled  with  red,  often  involvino- 
the  whole  thickness  of  the  cord,  and  occasionally  extending,  as  central 
softening,  through  a  considerable  vertical  extent.  Under  the  micro- 
scope the  usual  products  of  inflammation  are  seen,  sometimes  with 
hsematoidin  crystals.  Such  softening  may  occur  rapidly  in  severe 
local  concussion,  and  be  found  complete  a  few  weeks,  or  even  a  few 
days,  after  the  injury. J     On  the  other  hand,  in  some  cases  of  complete 

•  '  Brain,*  January,  1887. 

t  As  in  a  case  reported  by  Chucan  and  Wickham,  •  Prog.  Med.,'  1887.  Probably 
it  is  only  when  the  canal  is  previously  dilated  that  this  occurs,  as  it  may,  without 
serious  injury  to  the  nerve-tissue. 

J  Edmunds, '  Brain,'  vol.  vii,  p.  103;  Obersteiuer,  *Wien.  med.  Jahrb.,*  Bd.  iii, 
1879  J  Lochner, '  Bayer.  Aerztlich.  lat.-Bl.,'  1857,  No.  42;  Fromiiller,  ♦  Memorabilien,* 


654  SPINAL    CORD. 

paralysis,  no  lesion  of  the  cord  has  been  found,  either  with  the  nated 
eje  or  the  microscope,  a  few  days  after  the  injury.*  In  other  instances, 
in  which  the  cord  is  examined  some  weeks  ui  months  after  the  accident, 
the  signs  of  chronic  myelitis  are  found,  in  scattered  foci  or  more 
diffuse  tracts,  in  the  white  columns  or  grey  substance.  The  nerve- 
fibres  are  wasted,  and  the  connective-tissue  elements  are  increased  in 
quantity,  and  in  the  early  stages  there  may  be  a  leucocytal  infiltration 
about  the  vessels,  dilatation  of  the  capillaries,  and  minute  extravasa- 
tions, although  none  may  have  been  visible  to  the  naked  eye.  In  the 
anterior  cornua  the  motor  nerve-cells  may  be  damaged,  sometimes 
swollen  and  vacuolated,  or  shrunken,  and  the  anterior  root-fibres  may 
be  degenerated.  The  grey  matter  is  especially  apt  to  suffer  when  the 
enlargements  are  injured;  in  the  dorsal  region  the  change  may  be 
confined  to  the  white  columns.  Ill-defined  cavities  may  exist  in 
old  cases,  where  the  nerve-elements  have  perished.  The  usual 
ascending  and  descending  degenerations  may  be  found  above  and 
below  the  most  damaged  parts.  Occasionally  there  are  indications  of 
meningitis,  diffuse  or  disseminated,  and  sometimes  confined  to  the 
dura  mater. 

Symptoms. — The  effects,  immediate  and  remote,  of  injuries  of  the 
spinal  cord  extend  over  almost  the  whole  range  of  symptoms  of  cord 
disease,  and  their  variations  in  character  and  course  are  almost  infinite. 
It  is  therefore  neither  practicable  nor  necessary  to  do  more,  in  this 
place,  than  to  describe  their  general  characters.  According  to  the 
difference  in  coiirse,  we  may  divide  them  into  three  classes.  (1) 
Those  in  which  the  injury  causes  immediate  and  severe  paralysis, 
due  to  instant  damage  to  the  spinal  cord,  the  consequence  of  its 
laceration,  compression,  or  concussion.  (2)  Those  in  which  there  are 
at  first  either  no  symptoms  or  only  trifling  disturbance  of  function, 
but  in  which  grave  symptoms  come  on  a  few  days  or  weeks  after  the 
injury.  (3)  Those  in  which  there  are  no  early  symptoms,  or  only 
slight  and  transient  disturbance,  but  at  the  end  of  one  or  several 
months  symptoms  gradually  come  on,  often  such  as  indicate  disease 
of  some  definite  system  or  structure  of  the  cord,  degenerative  in 
nature. 

(1)  The  first  class,  in  which  the  injury  causes  instant  and  considerable 
disturbance  of  function,  includes  the  cases  in  which  the  cord  is  directly 
injured,  and  also  some  in  which  there  is  no  visible  sign  of  damage  if 
the  patient  dies  within  a  few  days.  In  the  former  class  there  is 
often  a  manifest  lesion  of  the  spine,  and  the  cases  are,  at  least  at  first, 
purely  surgical  in  their  practical  relations.  In  the  latter  the 
mechanical  influence  has  apparently  abolished  the  function  of  the 
nerve-elements.     If  such  patients  live  longer,  either  quick  recovery 

1870,  No.  12.  In  the  last,  described  further  on,  softening  was  found  tbirty*two 
hours  after  the  injury. 

•  Fischer,  *  Deut.  Zeitschr.  f.  Chirurg.,*  1883,  Bd.  xix. 


TRAUMATIC    LESIONS,  655 

ensues,  or  local  softening  from  disintegration  of  the  fibres  that  are 
most  damaged.  It  is  an  interesting  fact  that  concussion  may  thus 
derange  function.  The  absolute  integrity  of  structure  on  early  micro- 
scopical examination  shows  that  the  result  is  not  dxie  to  any  minute 
vascular  lesion.  The  effect  has  been  compared  by  Eeynolds,  not 
inaptly,  to  the  demagnetisation  of  iron  by  a  blow.  Doubtless  the 
influence  is  exerted  on  the  molecular  nutrition  of  the  nerve-elements, 
and  the  possibility  of  recovery,  or  the  subsequent  structural  dis- 
integration, depends  upon  the  degree  of  nutritional  damage.* 

The  symptoms,  in  these  cases  of  severe  and  immediate  effect,  are 
generally  those  of  complete  impairment  of  function.  When  the  injury 
is  direct  and  partial,  such  as  a  hemisection  by  an  incised  wound,  or  a 
partial  bruise  of  the  cord  by  a  spicidum  of  bone  being  driven  against 
it  (as  in  the  case  mentioned  at  p.  236),  the  effect  may  be  a  partial 
(e.  g.  one-sided)  derangement  of  function ;  but  in  most  other  cases 
there  is  abolition  of  all  the  conducting  functions  at  the  level  of  the 
injury.  There  is  complete  paraplegia,  motor  and  sensory,  with  loss 
of  power  over  the  sphincters.  The  symptoms  are  thus  those  of  a 
total  transverse  lesion  at  the  affected  level  (see  p.  269).  When  the 
cervical  region  is  injured,  the  arms  are  necessarily  affected  according 
to  its  seat.  Such  cases  often  illustrate  very  clearly  the  relation  of 
arm-function  to  the  cord.f  The  fibres  for  the  sympathetic  are  often 
implicated,  especially  those  for  the  iris,  causing  inequality  of  the  pupils. 
Eeflex  action  below,  abolished  at  first  by  shock,  usually  soon  returns 
and  becomes  excessive,  but  remains  absent  if  the  lumbar  enlargement 
is  damaged,  or  is  invaded  by  haemorrhage ;  it  may  be  again  lost  by 
the  downward  extension  of  secondary  inflammation,  which  is  equally 
effective  when  partial  (in  the  posterior  columns  or  grey  matter)  as 
when  total,  and  is  more  difiicult  to  detect  in  the  former  case.J  Loss 
of  consciousness  is  occasionally  produced  by  an  injury  which  does  not 
directly  involve  the  head,  as  in  the  case  mentioned  on  p.  656  ; 
vomiting  is  very  common  at  the  outset.  The  cases  of  direct  injury 
often  run  a  severe  and  rapid  course ;  and  when  the  secondary  inflam- 
mation spreads  downwards,  or  the  lower  part  of  the  cord  is  injured  (as 

•  These  conclusions  have  been  recently  formulated  more  definitely,  but  without 
essential  extension,  by  Schmaus,  partly  from  experiments  on  rabbits  ('Miincb.  med. 
Wochensch.,'  1890). 

+  See  especially  Thorburn,  *  Brain,'  January,  1887. 

J  This  is  the  explanation  of  most  cases  of  injury  above  fihe  lumbar  enlargement, 
with  permanent  loss  of  myotatic  irritability,  or  of  all  reflex  action,  as  in  those 
described  by  Bowlby  ('Lancet,'  1890,  i,  1071).  Such  descending  infla^iimation  must 
not  be  contused  with  the  descending  degeneration  in  the  pyramidal  tracts  which 
entails  myotatic  excess.  The  very  rare  exceptions  mentioned  on  p.  264,  in  which 
Buch  loss  exists  with  no  lesion  to  explain  it,  may  be  associated  with  the  fact  that 
myotatic  irritability  is  sometimes  lost  in  cases  of  cerebellar  tumour.  But  the  fact 
that  superficial  reflex  action  in  the  legs  is  never  lost  unless  the  lower  part  of  the  cord 
is  diseased,  is  one  of  the  most  certain  in  patliology.  Proof  of  such  descending 
inflammation  it  given  in  the  chapter  on  tumours  (p.  617,  note). 


656  SPINAL    CORD. 

is  frequently  the  case),  the  tendency  to  trophic  changes  in  the  sMn, 
and  to  the  occurrence  of  cystitis  and  pyelo-nephritis,  is  very  great. 
(Edema  of  vaso-motor  origin,  and  effusion  into  joints,  may  be  pre- 
sent in  the  early  stage.  If  the  cord  has  been  directly  injured  in  its 
whole  thickness,  survival  beyond  a  fortnight  is  rare.  When  it  is 
damaged  indirectly,  with  secondary  softening — in  what  may  be  termed 
concussion  myelitis — death  occurs  less  rapidly,  but  many  patients  die 
a.t  the  end  of  four  or  six  weeks,  while  in  those  who  survive  the  first 
two  months  slow  recovery  often  occurs.  Even  in  such  cases  death 
sometimes  occurs  very  quickly,  but  it  is  possible  that  there  is  then  a 
laceration  of  the  cord.  A  man,  whose  case  has  been  recorded  by 
Fromiiller,  was  struck  on  the  back,  at  the  level  of  the  third  dorsal,  by 
a  heavy  beam,  and  had  loss  of  motion  and  sensation  up  to  the  level 
of  the  nipples.  The  palsy  of  the  muscles  of  respiration  increased, 
and  he  died  from  asphyxia  at  the  end  of  thirty-two  hours.  At  the 
spot  struck  the  cord  was  reduced  to  a  pulp  for  2^  cm.,  without  any 
haemorrhage. 

The  following  case  illustrates  the  occasional  loss  of  consciousness 
when  there  is  no  evidence  of  any  cerebral  lesion.  A  heavy  weight 
fell  upon  a  man's  back ;  he  was  unconscious  for  two  days,  and  at  the 
end  of  that  time  the  legs  were  completely  paralysed,  and  continued  so 
for  three  months,  with  retention  of  urine.  Then  improvement  com- 
menced, in  the  left  leg  before  the  right,  and  in  the  latter  some  spasm 
developed.  At  the  end  of  six  months  he  could  walk  across  the  room 
on  crutches.  Improvement  continued,  but  at  the  end  of  two  years  his 
condition  became  stationary,  and  when  I  saw  him,  six  years  after  the 
accident,  he  could  only  walk  haK  a  mile ;  the  right  leg  was  still  weak 
in  all  parts,  and  a  foot-clonus  was  present  in  each  leg,  greater  in  the 
right  than  in  the  left. 

In  cases  in  which  the  lowest  part  of  the  back  receives  the  force 
of  the  blow,  as  when  a  severe  fall  ends  in  the  sitting  posture,  and  the 
sacral  region  is  strack,  the  nerves  of  the  cauda  equina  seem  often  to 
be  specially  damaged,  and  the  posterior  roots  in  greater  degree  than  the 
anterior.  The  effect  is  to  cause  loss  of  sensibihty  in  the  legs,  varying 
in  extent  and  seat  according  to  the  part  injured,  in  some  instances 
specially  great  about  the  anus,  in  others  extending  over  almost  the 
whole  of  the  legs,  in  others  again  it  is  chiefly  in  the  sciatic  area ;  all 
forms  of  sensation  are  usually  involved.  It  is  generally  accompanied 
by  some  muscular  paralysis  and  rapid  atrophy,  with  the  reaction 
of  degeneration,  especially  in  the  muscles  below  the  knees.  Rarely 
no  muscular  paralysis  exists;  but  there  is  loss  of  power  over  the 
sphincters  in  most  cases.  Reflex  action  is  lost,  and  trophic  changes 
are  readily  produced,  both  on  the  feet  and  over  the  sacrum.  The 
sensory  loss  is  often  permanent,  the  injury  to  the  nerve-roots  being 
apparently  too  severe  to  permit  recovery.  As  the  case  just  mentioned 
shows,  when  the  earlier  effects  of  the  injury  are  survived  and  improve- 
ment sets  in,  it  is  remarkable  how  long  this  may  continue,  and  how 


TRAUMATIC    LESIONS.  657 

considerable  a  degree  of  recoyerj  may  be  ultimately  attained,  it  may 
be  in  the  course  of  three  or  four  years. 

(2)  In  cases  of  the  second  class  initial  symptoms  are  absent  or  slight. 
After  the  accident  the  sufferer  may  not  imagine  himself  injured,  and 
may  be  able  to  walk  some  distance  without  inconyenience.  In  other 
instances  there  is  tingliag  ia  the  legs,  or  in  all  the  limbs,  immediately 
after  the  concussion,  sometimes  with  some  weakness  of  the  limbs, 
more  often  with  a  feeling  of  stiffness.  In  the  course  of  a  few  days 
grayer  symptoms  come  on,  usually  attended  with  spinal  pain  and 
tenderness,  sometimes  with  some  stiffness  of  the  back,  and  often  with 
pyrexia.  Tingling  in  the  limbs  increases  or  deyelops,  and  is  accom- 
panied by  weakness,  which  often  goes  on  to  complete  paralysis  in  the 
course  of  one  to  four  weeks.  The  symptoms  vary  in  their  character 
and  distribution,  according  to  the  position  of  the  morbid  process  and 
its  extent.  In  some  cases,  in  which  the  dorsal  region  is  most  affected, 
the  symptoms  are  those  of  simple  paraplegia,  usually  with  spasm,  and 
sometimes  with  early  contraction  of  the  muscles.  In  other  cases,  in 
which  the  grey  matter  of  the  enlargements  suffers,  there  is  scattered 
muscular  atrophy  in  the  limbs,  often  with  indications  of  the  degene- 
ratiye  reaction.  Tremor  in  the  limbs  is  conspicuous  in  some  instances. 
A  girdle-pain  in  the  trunk,  or  sense  of  constriction  in  the  limbs,  is 
very  common.  The  sphincters  generally  suffer,  and  the  tendency  to 
trophic  changes  is  usually  strong.  The  character  and  course  of  the 
symptoms  is  that  of  a  subacute  myelitis,  and  the  meagre  facts  of 
morbid  anatomy  leave  little  doubt  that  this  is  the  common  lesion. 
Thus  a  lady  was  severely  shaken  in  a  railway  collision.  She  seemed 
immediately  after  the  accident  to  have  suffered  no  injury,  but  in  a 
few  days  paraplegia  developed,  and  from  its  consequences  she  died 
six  weeks  after  the  accident.  Throughout  the  dorsal  region  of  the 
cord  I  found  indications  of  subacute  myelitis,  chiefly  in  the  white 
columns,  varying  in  its  extent  in  different  regions,  but  most  distinct 
in  the  pyramidal  tracts.*  Complete  motor  and  sensory  palsy,  up 
to  the  level  of  the  umbilicus,  developed  in  the  course  of  a  week, 
after  a  fall  downstairs  in  which  the  back  struck  successive  steps. 
Yet  the  patient  was  able  to  walk  immediately  afterwards.  The 
symptoms  in  these  cases  are  sometimes  unilateral.  A  man,  driving 
Tinder  a  low  archway,  leaned  back  to  save  his  head,  and  his  spine  was 
pressed  suddenly  against  the  sharp  edge  of  the  seat-back.  He  felt 
but  little  immediate  effect,  but  in  the  course  of  two  or  three  days 
complete  motor  palsy  of  the  left  leg  came  on,  accompanied  by  hyperses- 
thesia,  but  without  any  loss  of  sensation  either  in  that  leg  or  in  the 
other.  Power  slowly  returned  at  the  end  of  three  months,  and  was 
in  time  perfect.     In  some  cases  of  this  kind  the  symptoms  are  due 

*  In  the  face  of  such  a  case  as  this,  it  is  superfluous  to  discuss  the  question  raised 
by  some  writers  whether  or  not  the  cord  can  sufEer  concussion.  Moreover  it  is 
certain  that  such  an  effect  as  was  extreme  in  this  case^  may  and  must  often  occur  in 

slighter  degree. 

VOL.  I.  42 


658  SPINAL   CORD. 

not  to  direct  damage  to  tlie  cord,  but  to  its  compression  by  inflam- 
matory products  outside  it,  tlie  result  of  injury  to  the  vertebral 
column.  A  clergyman  was  thrown  from  his  horse,  and  there  was 
immediately  sufficient  weakness  of  the  legs  to  prevent  him  from 
walking;  this  subsequently  increased,  so  that  at  the  end  of  a  fort- 
night the  right  leg  was  completely  paralysed,  while  the  left  retained 
considerable  power.  There  was  a  girdle-pain  at  the  level  of  the 
umbilicus,  and  a  bedsore  formed,  but  he  slowly  improved,  and  regained 
the  power  of  standing  at  the  end  of  eight  months.  Such  symptoms 
may  come  on  weeks  after  the  concussion,  especially  when  the  effects 
of  this  are  so  slight  as  to  be  disregarded,  and  the  patient  leads  his 
usual  life. 

When  the  damage  involves  the  grey  matter,  the  extent  of  the 
muscular  wasting  varies  greatly.  It  seldom  affects  both  arms  and 
legs,  but  it  is  usually  irregular  in  distribution,  sometimes  wide- spread, 
sometimes  limited.  Thus  a  young  man  fell  from  a  horse  and  pitched 
on  the  head.  He  was  stunned,  and  on  recovering  consciousness  about 
two  hours  later  felt  "  pins  and  needles "  in  both  hands  and  pain  in 
the  back,  followed  by  swelling  of  the  neck  and  difficulty  in  moving  it. 
The  tingling  ceased,  but  was  followed  by  a  sense  of  oppression  about 
the  shoulders,  and  persistent  pain  in  the  cervical  region  of  the  spme. 
When  I  saw  him,  two  months  later,  there  was  some  weakness  of  the 
right  arm  and  wasting  of  the  two  outer  interossei,  and  of  that  part  of 
the  long  extensor  which  acts  on  the  two  outer  fingers,  with  loss  of 
faradic  and  preservation  of  voltaic  irritability. 

Sensory  symptoms  are  prominent  in  some  cases  of  this  class. 
Besides  the  pain  in  the  spine,  to  be  presently  mentioned,  pains  are 
often  felt  in  the  legs,  various  in  character ;  still  more  frequent  are 
spontaneous  sensations  of  tingling,  "pins  and  needles,"  and  the 
feelings  of  defective  sensibility  described  as  "numbness."  Various 
forms  of  hypersesthesia  are  also  common,  with  or  without  such  subjec- 
tive sensations.  This  may  exist  in  any  part,  in  the  soles,  feet,  thighs,  or 
not  infrequently  about  the  genitals.  In  the  latter  region,  and  about 
the  anus,  a  sense  of  coldness  is  often  complained  of.  In  these  regions 
there  may  be  a  distinct  defect  of  sensibility,  but  this  is  slighter  and 
less  frequent  than  in  the  cases  of  the  first  class.  Some  degree  of 
anaesthesia  to  touch  may  be  associated  with  increased  sensitiveness  to 
stronger  impressions.  These  sensory  disorders  may  exist  alone  or  be 
accompanied  by  motor  weakness.  Slight  unsteadiness  or  definite 
inco-ordination  is  present  in  some  cases,  usually  resembling  that  met 
with  in  ataxic  paraplegia. 

In  cases  which  survive  the  acute  stage  there  is  usually  slow  improve- 
ment, which  is  often  ultimately  very  great.  In  many  cases  recovery 
is  incomplete,  but  improvement  goes  on  for  years,  as  it  does  in  the 
cases  of  considerable  damage  to  the  cord  already  mentioned ;  the 
ultimate  degree  attained  is  commonly  much  greater  than  in  cases  of 
corresponding  character  and  severity  due  to  other  causes  than  injury. 


TRAUMATTO    LK SIGNS,  659 

Some  jjei'nictiient  symptoms  are  left,  chiefly  wT'eii  the  initial  deiixnge- 
ment  of  f  anction  has  been  severe,  and  has  lasted  for  some  time. 

(3)  In  the  cases  of  the  third  class,  chronic  symptoms  slowly  follow 
an  injury  at  an  interval  usually  of  some  months,  and  the  cases  have 
the  aspect  less  of  a  traumatic  lesion  of  the  cord  than  of  a  primary 
cord  disease,  the  relation  of  which  to  the  injury  is  rather  an  inference 
from  the  sequence,  coupled  with  the  absence  of  other  causes,  than  an 
obtrusive  fact,  as  in  the  cases  of  the  first  and  second  classes.  The  sym- 
ptoms are  usually  those  of  a  definite  system  disease  of  the  cord,  less 
frequently  those  of  a  disseminated  chronic  myelitis  of  irregular  distri- 
bution, still  more  rarely  those  of  chronic  focal  myelitis.  Primary  spastic 
paraplegia,  ataxic  paraplegia,  insular  sclerosis,  locomotor  ataxy,  and 
progressive  muscular  atrophy  are  the  diseases  which  most  frequently 
thus  result.  The  fact  that  an  injury  is  occasionally  the  cause  of  these 
maladies  has  been  already  mentioned  in  the  account  of  their  etiology, 
and  the  symptoms  of  such  cases  so  far  resemble  those  that  are  due  to 
other  causes  that  it  is  not  necessary  here  to  add  anything  to  their  his- 
tory as  already  given.*  We  must  assume  that  the  shock  to  the  nerve- 
elements  causes  a  slow  perversion  of  nutrition,  which  is  only  mani- 
fested by  disturbance  of  function  when  it  has  gradually  attained  a 
certain  degree.  Occasionally  other  causes  co-operate,  capable  of 
producing  degeneration.  Previous  syphilis,  or  neurotic  inheritance, 
can  sometimes  be  traced,  and  it  is  a  reasonable  assumption  that  the 
predisposition  thus  arising  may  assist  in  rendering  the  traumatic 
influence  efficient,  or  in  augmenting  its  effects.  Intermediate  cases 
connect  this  class  with  the  last,  cases  in  which  we  must  assume,  from 
indications  of  chronic  inflammation,  that  definite  injury  was  produced 
at  one  or  more  spots.  An  instance  of  this,  and  also  of  focal  myelitis, 
was  presented  by  a  man  with  permanent  spastic  paraplegia  and  an 
intense  girdle-pain  at  the  level  of  the  umbilicus.  These  symptoms 
commenced  three  months  after  a  jump  from  a  table,  and  shght 
unsteadiness,  with  pains  in  the  legs,  connected  the  jump  and  the 
paralysis. 

The  several  consequences  of  injury,  which  we  have  considered  as  oc- 
curring in  separate  form,  are  not  only  connected  by  intermediate  cases, 
but  are  also  sometimes  distinctly  combined.  Thus  an  actual  and 
immediate  lesion  of  the  cord  may  be  associated  with  an  early  and 
severe  increase  in  the  symptoms  from  the  development  of  subacute 
myelitis  in  other  parts.  The  traumatic  inflammation,  at  the  spot 
damaged,  often  attains  an  intense  degree,  and  runs  a  severe  course,  in 
consequence  of  the  influence  which  may  give  rise  to  such  inflammation, 
apart  from  a  positive  lesion ;  and,  in  both  cases,  the  damage  to  the 
cord  may  be  perpetuated  by  a  degenerative  tendency  It  is  very 
common,  for  instance,  for  an  iajury  to  the  cord,  in  those  who  have  had 

*  A  case  may,  however,  be  mentioned  in  which  typical  tabes  followed  slow  ly  a 
fall  on  the  back,  recorded  by  Barbour  ('  Journ.  of  Nervous  and  Mental  Discuses,* 
March,  1891,  Case  IV). 


660  SPINAL   COrtD. 

BypHlis,  to  cause  acute  symptoms  wMcb.  subside,  but  not  perfectly ; 
and  the  residual  disturbance  of  function  may  persist,  and  even  increase 
in  tlie  course  of  years,  in  consequence  of  the  influence  of  the  previous 
sypbilis  in  rendering  the  nerve-elements  prone  to  degenerate.  Again^ 
it  has  been  pointed  out  that  the  gouty  diathesis  is  an  unquestionable 
cause  of  myelitis,  and  often  of  the  grave  relapsing  and  spreading  dis- 
seminated variety.  A  concussion  of  the  cord,  which  causes  only  slight 
symptoms,  may  excite  the  occurrence  of  this  form  of  myelitis  in 
persons  thus  predisposed ;  the  symptoms  of  the  later  inflammation 
may  come  on  months  after  the  injury  (the  effects  of  which  ha/e 
generally  not  quite  passed  away),  and  are  sometimes  excited  by  some 
over-exertion,  or  slight  fresh  concussion  which  would  have  had  no 
effect  on  a  healthy  individual.  I  have  known  the  symptoms  of  insular 
sclerosis,  cerebral  and  spinal,  to  quickly  follow  traumatic  paraplegia. 
One  other  symptom  remains  to  be  considered,  which  is  common  to 
all  forms  of  traumatic  lesion  of  the  cord,  and  is  often  severe  and  per- 
sistent when  other  symptoms  are  slight — spinal  pain.  It  may  con- 
tinue for  years  after  other  symptoms  have  ceased,  and  probably 
depends  on  a  neuralgic  state  of  the  nerves  of  the  membranes  or  of 
those  of  the  vertebral  column ;  often,  probably,  the  pain  depends  on 
the  nerves  of  the  joints  and  ligaments  of  the  spine.  It  is  essentially 
a  traumatic  spinal  neuralgia.  The  pain  may  be  felt  at  one  or  more 
spots ;  when  severe,  it  often  extends  through  a  considerable  length  of 
the  spine,  and  sometimes  passes  up  to  the  occiput.  It  is  occasionally 
referred  to  the  sacrum,  and  may  there  have  the  character  of  a  sense 
of  weight  or  more  vague  discomfort.  The  pain  is  associated  with 
tenderness  of  the  spine,  usually  deep-seated,  chiefly  developed  at  the 
injured  part,  but  sometimes  present  also  at  other  spots.  It  may 
gradually  assume  the  features  of  a  true  neuralgia,  may  occur  in 
paroxysms,  and  be  iaduced  by  mental  and  other  influences  which  do 
not  act  directly  on  the  spine,  as  well  as  by  exertion,  posture,  and  other 
agencies  that  may  immediately  influence  the  affected  structures.  This 
condition  is  often  called  "  spinal  irritation." 

The  cause  of  traumatic  lesions  of  the  cord  often  acts  also  on  the 
braia.  A  cerebral  lesion  may  occur  from  the  violence  which  affects 
the  cord ;  the  cerebral  symptoms  then  co-exist  with  those  of  the  spinal 
lesion,  and  may  mask  the  latter  during  the  early  stage.  More  common, 
however,  is  functional  disturbance  of  the  brain,  the  result,  partly, 
perhaps,  of  the  physical  concussion,  but  chiefly  of  the  mental  shock 
which  a  serious  accident  necessarily  causes.  The  resulting  condition 
is  favorable  to  the  development  and  persistence  of  subjective  sensory 
symptoms.  Attention,  maintaiaed  by  concern,  has  a  powerful  in- 
tensifying influence  on  all  forms  of  nerve-pain,  and  certainly  aids  in 
keeping  up  the  pain  in  the  back,  and  even  the  tenderness  which 
follows  injuries  to  the  spine.  So  marked  is  the  influence  of  "  nervous- 
ness" on  the  subjective  symptoms,  that  it  has  been  even  maintained 
that  in  a  large  number  of  cases  of  concussion  of  the  spine  the 


THAUMATIO    LESIONS.  6G1 

symptoms  are  of  hysterical  origin.*  Well-marked  symptoms  of 
hysteria  are  sometimes  manifested  by  these  patients.  But,  on  the 
other  hand,  it  is  necessary  to  avoid  the  danger  of  over-estimating  the 
effect  of  mental  influence,  and  of  regarding,  as  entirely  due  to  this, 
symptoms  which  are  real,  and  are  merely  intensified  by  attention. 
The  danger  is  especially  great  in  cases  of  railway  injuries,  concerning 
which  an  unbiassed  judgment  is  not  easy  to  secure,  and  in  which,  when 
objective  symptoms  are  absent,  it  is  easy  to  minimise  suffering,  and. 
attribute  too  much  to  the  mental  condition.  The  sinister  influence  of 
litigation  on  the  intellect  may  be  traced  very  widely.f  I  believe  that 
it  is  rare  for  symptoms  to  be  purely  mental.  It  is  often  asserted  by 
those  employed  for  railway  companies  that  subjective  symptoms 
quickly  subside  when  the  sufferer's  "  claims  "  are  settled,  but  it  should 
be  remembered  that  mental  anxiety  is  a  potent  cause  of  diseases  of 
the  nervous  system,  and  must  be  strongly  opposed  to  recovery  from 
genuine  disorders.  The  occurrence  of  improvement  when  suspense  is 
at  an  end  is  thus  no  proof  in  itself  of  the  nature  of  the  case,  and  its 
significance  has  been  unquestionably  over-estimated  ;  moreover,  in  a 
great  many  individuals  whom  I  have  had  an  opportunity  of  observing 
long  after  they  had  received  their  "damages"  (as  the  expression 
curiously  runs)  this  subsidence  had  not  occurred,  and  even  the  "  sove- 
reign balm  "  of  substantial  compensation  has  appeared  to  do  very  Httie 
for  the  relief  of  the  sufferer. 

These  opinions  have  been  formed  from  a  study  of  cases  other  than 
those  that  involve  litigation,  in  which  no  elements  existed  to  bias  the 
judgment,  and  from  a  comparison  of  these  with  many  "  railway"  cases 
observed  apart  from  forensic  proceedings.  Those  who  desire  to  learn 
what  can  be  said  on  the  subject  of  "  railway  spines,"  as  they  have  come 
to  be  termed,  when  viewed  from  the  opposite  sides,  will  find  abundant 
material  for  consideration  in  the  writings  of  Erichsen  ('  On  Concussion 
of  the  Spine,'  London,  1875)  and  Clevenger  ('  Spinal  Concussion,  or 
Erichsen's  Disease,'  Philadelphia,  1889)  on  the  one  side,  and  of  Page 
('  Injuries  of  the  Spine  and  Spinal  Cord,'  London,  2nd  ed.,  1885)  on  the 
other.  Scattered  papers  by  J.  J.  Putnam,  Walton,  Spitzka,  Buzzard, 
and  others,  more  or  less  instructive,  will  be  found  epitomised  in 
Clevenger's  work,  where  indeed  is  collected  the  pith  of  almost  all  that 
has  been  written  on  the  subject. 

Diagnosis. — The  chief  points  in  the  diagnosis  of  traumatic  lesions 
of  the  cord  have  been  already  incidentally  considered.  Immediate 
symptoms  may  be  due  to  laceration,  haemorrhage,  or  to  simple  con- 
cussion, and  the  diagnosis  between  these  is  not  always  possible  at 
first.      If  there  are  immediate  symptoms  of  a  partial  lesion,  these 

•  J.  J.  Putnam,  '  Boston  Med.  and  Surgical  Journal/  1883,  Sept.  6th. 

t  Not  many  years  ago  it  was  customary  for  the  "  experts"  who  gMve  evidence  on 
behalf  of  railway  companies  to  deny  that  the  spinal  cord  could  he  injured  if  the  legs 
were  unwasted.  Although  the  opinion  dare  not  now  be  expressed,  its  significance  is 
not  without  analogies  at  the  present  day. 


632  SPINAL    COED. 

indicate  direct  injur  f,  wliile  tlie  rapid  snlDsidence  of  tlie  disturbance 
of  function  render?  simple  concussion  probable,  and  excludes  any 
considerable  direct  injury.  The  later  development  of  paralysis  in- 
dicates myelitis,  w  Jess  tbere  is  evidence  of  considerable  irritation  of  tbe 
nerve-roots  at  a  ''ertain  level,  vrliicli  suggests  inflammation  outside  the 
cord,  and  perhar  s  even  outside  the  dnra  mater.  The  greatest  diagnostic 
difficulty  is  p.esented  by  the  cases  just  mentioned,  in  which  the 
sjTnptoms  ar;  subjective,  and  anxious  attention  has  been  long  given 
to  the  local  ''Jiscomfort.  The  chief  elements  in  the  diagnosis  of  these 
cases  have  ^/een,  however,  already  indicated.  It  is  important  to  search 
for,  and  tr  give  due  weight  to,  any  symptoms  beyond  the  simple  spinal 
pain.  SI  -ght  "  tingling  "  or  "  creeping  "  sensations  may  be  of  cerebral 
and  "  fu'ictional"  origin,  but  apersistent  sensation  of  "  pins  and  needles" 
rarely  's  of  that  nature.  A  definite  sense  of  constriction  is  also 
stronjT.y  suggestive  of  organic  disease,  and  so  is  a  well-marked 
differ' 3nce  in  the  power  of  the  muscles  on  the  two  sides.  The  latter  is 
of  V^st  significance  if  the  excess  is  slight,  general,  and  on  the 
riq^it  side — of  much  greater  significance  if  the  diminution  is  partial, 
p^id.  affects  only  certain  groups  of  muscles,  such  as  the  flexors  of  the 
'jip  and  knee,  or  the  peronei.  Any  impainnent  of  power  over  the 
bladder  or  rectum  is  of  great  diagnostic  importance ;  loss  of  sexual 
power,  on  the  other  hand,  is  of  little  value,  since  this  function  is 
readily  depressed  by  mental  anxiety  and  preoccupation.  A  slight 
change  in  reflex  action  is  most  significant  when  it  is  partial.  A  foot- 
clonus  or  rectus-clonus  is  strong  presumptive  evidence  of  organic 
mischief.  A  slight  excess  of  the  knee-jerk  is  of  little  value ;  although 
it  probably  always  indicates  some  changes  in  the  nutrition  of  the 
spinal  cord,  it  does  not  indicate  structural  disease.  In  all  cases  it 
should  be  remembered  that  the  absence  of  any  common  symptom 
is  of  far  slighter  significance,  as  evidence  of  integrity  of  the  cord, 
than  is  the  presence  of  that  symptom  as  evidence  of  disease.  It 
may  seem  superfluous  to  insist  on  a  consideration  so  elementary,  but 
it  is  still  possible,  as  experience  jDroves,  for  a  medical  witness  to  assert 
in  a  cou.rt  of  law  that  a  claimant's  spinal  cord  cannot  have  been  injured 
because  some  symptom  is  absent,  the  presence  of  which  would  be 
important. 

Peognosis. — Immediately  after  an  accident  a  cautious  prognosis 
should  be  given,  even  if  the  symptoms  are  slight,  on  account  of  the 
possibility  that  grave  disturbance  may  develop  in  the  course  of  a  few 
days.  In  developed  cases  the  prognosis  must  in  general  be  guided 
by  the  same  considerations  as  those  which  determine  our  estimation  of 
the  probable  course  of  symptoms  of  similar  character  and  severity 
due  to  spontaneous  myelitis.  To  this  there  are,  however,  two  general 
exceptions.  First,  the  danger  of  death,  if  any  exists,  is  greater  in 
traumatic  cases  than  in  others,  as  long  as  the  symptoms  are  increasing. 
Secondly,  if  there  is  no  danger  to  life,  or  such  danger  has  passed,  the 
prospect  of  improvement  is  distinctly  greater  than  in  a  case  of  similar 


TEAUMATIO   LESIONS.  6G3 

features  "but  of  non-traumatic  origin.  If  the  symptoms  are  sliglit  or 
moderate  in  degree  approximate  recovery  may  be  anticipated,  although 
Blight  symptoms  often  endure  for  a  very  long  time.  Indeed,  in  many 
cases  recovery,  although  approximate,  is  not  perfect.  The  patient  is 
never  quite  as  strong,  never  becomes  quite  as  capable  of  exertion, 
as  before  the  injury.  A  cautious  prognosis  should  also  be  given 
whenever  there  is  the  late  and  gradual  onset  or  increase  of  symptoms 
that  suggests  a  degenerative  process.  Such  degeneration  presents  far 
less  tendency  to  arrest  or  subsidence  than  do  the  earlier  lesions.  As 
a  rule  the  sooner  symptoms  occur  the  better  is  the  prospect  of  ultimate 
improvement  or  recovery,  provided  they  are  not  so  severe  as  to  be 
incompatible  with  life. 

Treatment. — The  early  treatment  of  these  cases,  and  much  of  the 
later  treatment  of  those  in  which  the  spinal  column  is  injured,  is 
purely  surgical.  The  points  of  medical  character  alone  need  special 
mention,  and  many  of  these  have  been  anticipated  in  the  preceding 
pages.  In  all  cases  in  which  spinal  symptoms  are  present  im- 
mediately after  an  injury,  however  slight  those  symptoms  may  be, 
absolute  rest  should  be  insisted  on  for  some  days  or  weeks,  according 
to  the  severity  of  the  early  symptoms.  This  is  necessary  on  account 
of  the  secondary  inflammation,  which,  as  we  have  seen,  so  often  occurs. 
The  treatment  of  developed  symptoms  must  be  conducted  on  the  same 
general  principles  as  in  cases  of  myelitis;  the  details  need  not  be 
here  repeated.  If  there  is  muscular  wasting,  it  is  important  that  the 
nutrition  of  the  muscles  should  be  maintained  by  electrical  stimula- 
tion, since  a  very  considerable  amount  of  ultimate  recovery  may  be 
anticipated,  and  it  is  important  to  keep  the  muscular  tissue  as  far 
as  possible  in  a  condition  to  respond  to  the  nerve-power  when  this 
returns.  If  there  is  reason  to  believe  that  there  is  inflammation  of 
the  membranes,  or  inflammatory  effusion  outside  the  cord,  com- 
pressing it,  mercury  may  be  given,  but  this  condition  is  probably 
much  more  rare  than  might  be  anticipated.  The  infliience  of 
mercury  on  inflammation  of  the  substance  of  the  cord  is  doubtful. 
The  chief  element  in  treatment  is  patiently  to  permit  time  to  do  its 
work,  and  the  tissues  to  slowly  regain  such  integrity  of  structure  and 
function  as  is  possible;  meanwhile  preserving  the  patient  from  all 
influences  likely  to  interfere  with  the  process  or  to  set  up  other  mischief, 
such  as  cystitis  or  bedsores,  which  would  entail  fresh  danger.  There 
is,  however,  one  therapeutic  measure  that  is  of  unquestionable  value 
in  the  treatment  of  the  later  stages,  especially  of  the  cases  in  which 
the  recurring  symptoms  suggest  a  relapsing  myelitis  as  the  sequel  of 
injury ;  and  that  is  the  repeated  application  of  a  mild  "actual  cautery" 
on  each  side  of  the  spine  opposite  the  affected  region.  Several 
applications  should  be  made,  with  or  without  an  anaesthetic.  It  is 
not  desirable  to  lessen  the  pain  by  cocaine,  because  this  is  likely  to 
interfere  with  the  influence  of  the  proceeding,  the  beneficial  character 


664  SPINAL  jCORD. 

of  whicli  is  -andoubted.*  The  degenerative  sequelse  of  injuries  to  tlie 
cord  need  tlie  same  treatment  as  the  similar  degenerations  that  occur 
apart  from  traumatic  influences.  Whenever  there  is  evidence  of  dis- 
placement of  the  bones,  or  reason  to  suspect  that  the  cord  is  compressed 
by  fractured  fragments,  or  even  by  products  of  secondary  inflammation 
outside  it,  the  propriety  of  trephining  the  spine  needs  to  be  considered. 
The  problem  is  chiefly  surgical,  but  the  fact  that  it  will  probably  not 
be  necessary  to  open  the  dura  mater  increases  the  desirability  of 
giving  the  patient  this  chance  of  relief.  If  needed,  the  sooner  the 
measure  is  adopted,  when  improvement  has  ceased,  the  better. 

The  treatment  of  the  neuralgic  condition  of  spinal  pain  and  tender- 
ness, which  so  often  succeeds  injury,  is  frequently  difficult.  Counter- 
irritation  is  often  useful,  either  by  the  actual  cautery,  blisters,  iodine, 
or  repeated  sinapisms.  Of  sedatives,  Indian  hemp  is  most  effective, 
next  to  morphia,  which  should  be  used  as  seldom  as  possible.  Hypo- 
dermic injections  of  cocaine  may  be  tried.  When  all  active  mischief 
is  over,  and  the  pain  has  become  purely  neuralgic,  it  is  often  neces- 
sary to  encourage  the  patient  to  neglect  it  in  some  degree,  and  to 
exert  himself  in  spite  of  it,  while  avoiding  whatever  increases  it  in 
considerable  degree  and  for  a  considerable  time.  At  the  same  time 
extreme  care  should  be  observed  by  all  persons  who  possess  the 
constitutional  states  above  mentioned,  predisposing  them  to  myelitis, 
&c.  This  is  especially  necessary  when  any  symptoms  persist,  and 
often  difficult  to  secure,  except  by  uncompromising  insistence,  in  those 
who  have  been  accustomed  to  a  life  of  active  exertion. 


FUNCTIONAL   AND    NUTRITIONAL   DISEASES. 

Functional  Diseases. 

Very  little  is  known,  though  much  is  heard,  of  functional  diseases 
of  the  spinal  cord.  It  is,  indeed,  open  to  doubt  whether  there  are  any 
morbid  states  which  can  accurately  be  thus  designated;  as  was 
pointed  out  in  the  Introduction,  most  morbid  states  thus  described 
are  either  due  to  disturbance  of  cerebral  functions,  or  are  the  result 
of  changes  in  the  nutrition  of  the  elements  of  the  cord.  The  deranged 
function  may  be  an  expression  of  altered  nutrition,  as  it  is  of  altered 
structure,  but  such  cases  can  only  be  termed  "  functional "  by  a  loose 
misuse  of  words.  We  have  an  example  of  the  transference  of  our 
conceptions  of  functional  derangements  from  the  brain  to  the  cord, 
in  the  fact  that  hysterical  paraplegia  is  often  regarded  as  a  functional 
affection  of  the  cord  because  the  symptoms  have  the  same  distribution 
as  those  of  organic  diseases  of  the  cord ;  but  a  little  consideration 

•  For  first  directing  my  attention  to  the  fact,  and  the  evidence  supporting  it  in 
these  cases,  I  am  indebted  to  Dr.  John  Anderson. 


FUNCTIONAL    DISEASES.  665 

will  stow  that,  in  a  case  of  purely  liysterical  paraplegia,  the  morbid 
functional  condition  is  cerebral ;  the  brain-centres  which  act  on  the 
legs  are  at  fault,  but  the  condition  of  the  functions  of  the  cord  itself 
may  be  absolutely  normal.  The  spinal  motor  centres  are  in  a  state  of 
inactivity  because  the  related  cerebral  centres  are  inactive,  but  this  is 
no  more  a  diseased  condition  of  the  cord  than  is  its  corresponding 
functional  state  during  physiological  rest.  Hysterical  paraplegia  will 
be  described,  with  other  palsies  of  like  origin,  in  the  chapter  on 
hysteria — a  malady  for  which  the  term  "  functional  "  is  often  employed 
in  various  ways,  as  a  convenient  euphemism. 

At  the  same  time,  it  must  be  remembered  that  there  is  no  sharp 
line  of  demarcation  between  functional  derangement  and  nutritional 
changes.  As  already  mentioned,  no  functional  state  can  exist 
without  leaving  behind  it  some  corresponding  change  in  the  finer 
molecular  nutrition  of  the  structures ;  and  if  functional  derangemeni 
of  lower  structures  results  from  that  of  higher  cerebral  centres,  and 
is  maintained  for  long,  the  change  in  nutrition  that  results  may  be 
considerable,  and  may  be  so  definite  as  to  persist  even  after  its  cause 
has  ceased  to  act.  Moreover  the  general  enfeeblement  of  defective 
nutrition  of  the  nerve-elements  is  a  potent  cause  of  this  functional 
disturbance,  and  at  once  facilitates  and  augments  such  effects.  The 
process  may  be  observed  in  many  cases  not  only  of  hysteria,  but 
also  of  other  derangements  of  the  cerebral  functions,  and  it  is  seen 
also  in  lower  centres  as  an  effect  of  pain. 

Isolated  disturbance  of  functional  centres  in  the  cord  does,  however, 
sometimes  occur.  It  is  seen  in  the  conditions  of  inhibition  which  are 
designated  "reflex  paralysis."  Such  palsies  were  once  thought  to  be 
common  and  persistent,  but  it  has  been  proved  that  many,  and  it  is 
probable  that  all,  cases  of  considerable  and  prolonged  palsy,  formerly 
thought  to  be  of  reflex  origin,  are  due  to  organic  disease,  either 
primary  in  the  cord  or  secondary  to  an  ascending  inflammation  of 
nerves.  But  transient  paralysis  sometimes  occurs,  which  cannot  be 
otherwise  explained  than  as  an  inhibition  of  a  spinal  centre,  due  to 
peripheral  irritation.  Such,  for  instance,  is  the  curious  inability  to 
pass  urine  which  sometimes  follows  an  operation  on  the  anus, — the 
division  of  a  fistula,  for  instance,  or  the  removal  of  haemorrhoids. 
The  inability  may  continue  absolute  for  several  days.  Transient 
weakness  of  one  ai*m  is  said  sometimes  to  follow  an  operation  for 
empyema  (Lepine),  but  the  fact  that  the  weakness  may  be  attended 
by  choreoid  movements  in  both  arm  and  leg  of  one  side  (Weil) 
makes  it  probable  that  the  influence  is  exerted  on  a  cerebral  rather 
than  on  a  spinal  centre.*  Considerable  paraplegia  was  thought  to  be 
sometimes  a  reflex  effect  of  disease  of  the  bladder,  or  of  a  calculus  or 
other  organic  disease  in  the  kidney,  but  it  is  probable  that  such  cases 
are  always  of  organic  nature.     Ascending  neuritis,  reaching  the  cord, 

*  Paralysis  and  wasting  of  serrati  magni  and  infra-spinati  muscles  have,  how- 
ever, followed  double  empyema  (Cayley,  'Clin.  See.  Trans.,'  1898,  vol.  xxxi). 


666  SPINAL    COHD. 

has  also  been  proved  to  be  tlie  cause  of  lasting  symptoms  in  many 
«ases  supposed  to  be  of  reflex  origin.  In  one  important  class  of  cases 
in  wMcli  functional  disturbance  of  the  cord  is  purely  secondary  to  a 
morbid  state  of  the  brain,  organic  or  not,  as  has  been  already  men- 
tioned, the  expression  in  the  limbs  of  all  deranged  functions  of  the 
brain  is  through  the  spinal  cord ;  but  such  disturbance  of  the  cord  is 
purely  secondary,  and  ceases  when  the  cerebral  centres  resume  their 
normal  state ;  or,  if  it  is  continued  longer,  this  is  merely  for  a  brief 
space  of  time,  until  the  disturbed  structures  can  regain  their  normal 
state.  Thus,  iii  idiopathic  epilepsy,  the  convulsion  is  produced  through 
the  agency  of  the  spinal  structures,  and  their  over-action  may  leave 
them  so  exhausted  that  the  reflex  functions  are  for  a  short  time 
abolished.  But  such  conditions  cannot  be  regarded  as  coming  into 
the  category  of  functional  disease  of  the  cord. 

The  nearest  approach  to  such  disease  is  presented  by  certain  forms 
of  spasmodic  disorder,  such  as  transient  fixed  spasm,  catalepsy,  and 
certain  forms  of  tonic  spasm,  which  depend  proximately  on  spinal 
centres ;  and  by  some  varieties  of  neuralgic  and  other  painful  affec- 
tions or  dysaesthesise,  in  which  the  sensory  elements  of  the  cord  are 
deranged  in  action.  But  we  can  seldom  feel  sure  that  the  symptoms 
in  the  former  class  are  dependent  on  a  primary  derangement  of  the 
cord ;  more  often  they  are  clearly  secondary  to  disturbance  of  cerebral 
centres,  which  may  indeed  co-exist,  and  the  cases  are  better  described 
among  the  general  functional  affections  of  the  nervous  system ;  while 
those  of  the  second  class  have  features  and  alliances  which  make 
it  more  convenient  to  describe  them  among  the  forms  of  neuralgia. 

In  another  series  of  cases,  derangement  of  the  motor  functions  of 
the  spinal  cord  is  secondary  to  pain,  generally  that  which  has  such 
a  seat  that  it  is  increased  by  movement ;  sometimes  pain  that  is  not 
related  to  movement,  but  is  so  intense  as  to  act,  by  mere  severity,  on 
related  structures.  It  may  be  in  the  spine  itself,  in  the  trunk,  or  in 
the  limbs.  Over-action  of  the  motor  centres  and  spasm  may  be  the 
result,  or  the  centres  may  be  inhibited,  causing  a  pseudo-paralysis, 
which  may  be  real,  but  often  blends  with  a  voluntary  indisposition  to 
move  the  parts,  lest  pain  be  produced.  Spasm  is  generally  brief,  and 
the  reflex  result  of  some  sudden  pain,  as  is  often  seen  in  the  case  of 
the  "  lightning  pains  "  of  tabes.  When  more  prolonged  contracture 
resixlts,  as  in  cases  m  which  hysterical  contractTire  follows  some 
painful  affection  of  a  limb,  the  co-ox3eration  of  cerebral  centres  cannot 
be  excluded.  The  inhibitory  influence  is  strikingly  seen  in  the 
immobility  of  one  half  of  the  thorax  in  pleurodynia ;  and  in  brachial 
neuritis  the  effect  may  render  it  impossible  to  say  whether  or  not 
there  is  real  motor  weakness.  Some  forms  of  spasm  may  perhaps  be 
due  to  primary  disorder  of  the  spinal  centres,  such  as  the  peculiar 
cramp  in  the  hands,  coming  on  during  sleep,  that  is  described  in 
Vol.  II  as  "  nocturnal  tetany,"  and  the  startings  of  the  Hmbs  on  going 
to  sleep ;  but,  for  the  most  part,  these  spasmodic  a,ffections  are  either 


FUiNCTlONAL    DISEASES.  667 

eecondary  to  morbid  states  of  tlie  cerebral  motor  centres,  or  form  part 
of  diseases  of  wider  range  in  connection  with  whicb  they  are  described. 

Other  cases  which  may  be  regarded  as  functional  are  those  in 
which  symptoms,  commonly  subjective  in  character,  result  from 
some  morbid  blood-state.  The  conditions  which  most  frequently 
have  this  effect  are  gout  and  diabetes.  Occasionally  there  is  defi- 
nite failure  of  power,  lasting  for  a  few  days  or  weeks,  without 
objective  symptoms,  and  passing  away.  But  the  most  common 
symptoms  from  this  cause  are  sensory  and  subjective, — feehngs 
of  tingling  and  formication  in  the  legs,  dull  aching,  and  sometimes 
actual  pain ;  this  is  usually  transient,  but  occasionally  continues  for 
some  days  or  weeks,  various  in  position,  but  in  gouty  cases  often  felt 
in  the  heels.  Such  symptoms  due  to  morbid  blood-states  occur  chiefly 
during  the  second  half  of  life.  Those  of  gouty  origin,  like  other 
symptoms  of  the  same  class,  are  especially  common  in  persons  wha 
inherit  a  tendency  to  gout,  but  have  not  suffered  from  attacks  of 
definite  arthritis. 

In  the  diagnosis  of  functional  disorders  the  first  element  is  the  ex- 
clasion  of  any  sign  of  positive  disease,  and  the  second  is  the  discovery 
of  some  morbid  state  capable  of  giving  rise  to  the  functional  derange- 
ment, or  of  associated  symptoms  unequivocally  functional  in  character. 
The  first  of  these  is  that  of  primary  and  paramount  importance, 
which,  ahke  in  practice  and  reasoning,  should  precede  the  others. 
The  various  points  involved  in  the  diagnosis  of  hysterical  affections 
are  described  in  the  chapter  on  that  disease  in  Vol.  II ;  one  only  need 
be  mentioned  here — ^the  importance  which  attaches  to  the  state  of 
myotatic  irritability  in  the  legs,  and  the  great  difficulty  in  ascertaining- 
it  presented  by  many  of  these  cases.  Permanent  excess  shows  that 
there  is  more  than  functional  derangement;  although  it  does  not 
exclude  the  latter,  it  indicates  changes  in  nutrition,  and  will  be  re- 
turned to  presently.  The  cases  in  which  the  knee-jerk  is  apparently- 
absent  present  a  special  difficulty.  It  is  never  really  absent  in  func- 
tional disorder  (except  when  the  centres  are  exhausted  by  violent 
convulsion),  any  more  than  it  is  in  health.  Failure  to  obtain  it  is 
due  to  the  inabiHty  of  the  patient  to  relax  the  muscles,  which  may 
render  attempt  after  attempt  futile,  tmtil,  in  some  fortunate  moment, 
relaxation  is  secured  and  a  characteristic  jerk  is  obtained.  The 
expedients  that  facilitate  the  attempt  have  been  already  mentioned 
(p.  21),  and  the  subject  has  been  sufficiently  discussed  in  the  account 
of  the  diagnosis  of  tabes. 

The  treatment  of  functional  disorders  is  that  of  the  morbid  states 
to  which  they  are  due,  or  of  which  they  form  part,  and  does  not  need 
special  description.  That  of  the  symptoms  due  to  toxic  influences  is 
necessarily  the  removal  of  the  condition  which  causes  them,  but  they 
may  be  to  some  extent  relieved  by  sedatives,  of  which  bromide  of 
potassium,  cocaine,  Indian  hemp,  cimicifuga,  and  small  (7  or  10  gr.) 
doses  of  chloralamide  are  the  most  effective. 


€68  SPINAL    CORD. 


NXJTEITIONAL   DISEASES. 

Among  the  cases  often  classed  as  functional  diseases  of  the  spinal 
cord  are  some  in  which  objective  symptoms  of  deranged  function, 
slight  in  degree  but  definite  in  character,  persist  for  months,  years,  or 
for  life.  They  commence  chiefly  in  those  in  the  early  and  middle  period 
of  adult  life,  and  are  more  common  in  women  than  in  men.  Such 
symptoms  are  inability  to  walk  more  than  a  short  distance  without 
fatigue,  impaired  nutrition  of  the  legs,  slight  increase  of  myotatic 
irritability,  often  associated  with  pain  in  the  back,  aching  in  the  legs 
(sometimes  amounting  to  actual  pain),  and  various  spontaneous  sensa- 
tions of  tingling,  "  pins  and  needles,"  formication,  and  the  like.  There 
is  often  aching  in  the  legs  at  night,  especially  when  the  patient  lies  on 
the  back,  and  hence  probably  due  to  passive  accumulation  of  blood  in 
the  spinal  vessels.  Such  symptoms  occur  especially  in  those  of  neTurotic 
disposition,  who  often  present  other  signs  of  the  nervous  weakness  that 
it  is  now  fashionable  to  term  "  neurasthenia."  In  many  cases,  if  the 
history  of  such  symptoms  is  traced,  they  will  be  found  to  date  from 
some  definite  exciting  cause,  from  an  attack  of  acute  illness,  such  as 
typhoid  fever  or  acute  rheumatism,  from  pregnancy,  a  fall,  over- 
exertion, and  the  like,  or  to  have  come  on  during  a  period  of  general 
or  nerve  weakness,  anaemia,  anxiety,  &c.  Enfeeblement  of  the 
nervous  system  often  results,  and  the  impaired  nutrition  of  the  cord 
may  be  only  part  of  a  similar  general  state.  A  slight  increase  in 
myotatic  irritability  is  exceedingly  common,  enough  to  permit  the 
knee-jerk  to  be  obtained  by  tapping  the  depressed  patella ;  but  there 
is  rarely  a  distinct  clonus,  at  most  only  two  or  three  jerks,  quickly 
ceasing.  We  cannot  conceive  that  symptoms  so  persistent  can  depend 
on  any  mere  functional  derangement ;  it  is  probable  that  they  depend 
upon  changes  in  the  finer  nutrition  of  the  nerve-elements,  too  slight 
to  be  detected  by  the  microscope,  but  causing  a  corresponding  and 
persistent  alteration  of  function.  We  have  seen  that  the  termination 
of  the  upper  segment  of  the  motor  path  is  probably  that  structure  of 
the  cord  which  has  least  nutritional  stability,  and  is  therefore  most 
susceptible  of  nutritional  derangement.  Its  degeneration  is  the 
apparent  cause  of  persistent  excess  of  myotatic  irritability,  and  hence 
we  can  understand  the  frequency  with  which  there  is  such  an  excess  in 
these  cases.  There  are  probably  gradations  between  such  conditions 
and  actual  striictural  disease  in  which  minute  examination  reveals 
visible  alteration;  and  there  are  certainly  gradations  between  the 
symptoms  above  described,  and  those  in  which  the  derangement  of 
function  is  so  considerable  and  special  that  the  case  must  be  regarded 
as  one  in  which  actual  disease  exists,  of  one  or  other  of  the  types 
described  in  the  preceding  pages.  We  have  already  had  occasion  to 
consider  these  nutritional  changes  as  probably  underlying  the  con- 
dition of  arthritic  muscular  atrophy,  and  we  have  seen  that  the  altera- 


NUTKITIUNAL    DISEASES.  669 

tions  in  spinal  nutrition  in  tliat  disease  are  apparently  tie  result  of 
the  impressions  on  tlie  periplieral  nerres.  In  women  such  a  con- 
dition is  often  associated  with  uterine  or  ovarian  pain,  and  with  sacral 
pain  apparently  of  uterine  origin.  It  is  possible  that  the  condition 
we  have  been  considering  is  sometimes  secondary  to  such  uterine  pain, 
which  acts  in  a  manner  somewhat  analogous  to  that  in  which  joint 
inflammation  acts,  and  that  this  is  the  explanation  of  many  of  the 
cases  in  which  a  reflex  disturbance  of  the  functions  of  the  cord  has 
been  supposed  to  be  of  uterine  origin.  The  condition  is  also  often 
associated  with  spinal  pain  and  tenderness,  which  may  have  a  similar 
influence. 

In  the  diagnosis  of  these  nutritional  affections  of  the  spinal  cord  it 
is  important  to   remember  that  they  may  be  closely  simulated  by 
analogous  changes  in  the  peripheral  nerves,  constituting  the  slighter 
degrees  and  forms  of  polyneuritis.     The  chief  points  in  the  distinction 
have  been  described  in  the  account  of  that  disease.     It  is  in  the 
parenchymatous  forms,  and  especially  in  the  sensory  varieties,  that  the 
danger  of  confusion  is  apt  to  arise.     Symmetry  in  the  distribution  of 
the  subjective  sensations,  or  positive  anaesthesia,  and  their  localisation 
in  the  extremities,  are  the  most  important  features  of  neuritic  change. 
Connected  with  this  distinction  is  another,  of  not  less  importance. 
Nutritional  changes,  equally  with  the  simpler  functional  derangement, 
may  be  the  result  and  expression  of  the  action  of  some  toxic  blood- 
state,  and  aU  that  has  been  said  ia  connection  with  mere  alteration  in 
function  applies  also  to  the  class  now  under  consideration.     Toxic 
influences  cannot,  indeed,  act  for  long  without  leading  to  changes  in 
nutrition,  which  may  speedily  attain  a  degree  that  amounts  to  visible 
structural   disease.    A  search  for  such  an  agency  should  never  be 
neglected  in  any  case  in  which  the  symptoms  have  not  followed  an 
adequate  cause,  and  do  not  form  part  of  a  general  state  of  the  same 
nature.     But  it  must  be  remembered  that  the  peripheral  nerves  are, 
as  a  rule,  more  susceptible  to  toxic  influences  than  is  the  spinal  cord, 
and  this  fact  gives  additional  weight  to  any  indications  that  it  is  from 
these  that  the  symptoms  proceed.     Lastly,  symptoms  suggestive  of 
mere  changes  in  the  finer  nutrition  of  the  nerve- elements  are  fre- 
quently the  earliest  indications  of  organic  disease  of  one  or  another 
of  the   types  already  described,  and  in  aU  recent  and  progressive 
cases  their  general  character  and  aspect  must  be  carefully  considered, 
and  compared  with  the  known  features    of    the    various    organic 
diseases. 

The  cases  with  such  slight  but  persistent  symptoms  as  have  been 
just  described  vary  much  in  their  features,  and  may  present  all  sorts 
of  slight  symptoms,  in  the  utmost  diversity  of  degree  and  combina- 
tion. To  consider  them  in  detail  would  be  to  describe  an  indefinite 
series  of  individual  cases,  no  two  'of  which  are  identical.  The  precise 
degree  and  manner  in  which  the  nerve-elements  suffer  may,  it  is 
evident,  be  almost  infinitely  various.     Their  corresponding  manifesta- 


<370  SPINAL    COED, 

tions  present  features,  combiaations,  and  gradations  tliat  entirely 
baffle  an  attempt  to  designate  tliem,  or  even  to  perceive  definite  types 
about  wliicli  they  can  be  grouped.  Tliese  are  some  of  the  cases  of 
disease  "nliich  exemplify  the  failure  of  types  in  practical  work,  the 
error  involved  in  the  attempt  to  give  names,  and  the  paramount 
importance,  in  the  process  of  diagnosis,  of  considering  what  morbid 
condition  the  symptoms  indicate,  and  of  dealing  with  each  case  as  a 
new  problem,  sui  generis,  whether  or  not  it  is  ultimately  found  to  be 
one  of  a  familiar  series.  This  method,  moreover,  conducts  the 
practitioner  at  once  to  the  morbid  processes  that  need  treatment, 
and  should  determine  the  character  of  his  efforts  to  alter  that  which  is 
at  fault. 

It  is  unfortunately  not  common  for  the  indications  of  nutritional 
disturbance  of  the  cord,  when  they  have  been  long  established,  to  pass 
away  altogether,  although  some  degree  of  improvement  can  often  be 
secui'ed;  and  in  many  cases,  especially  in  those  of  brief  duration, 
approximate  restoration  to  a  normal  state  may  take  place. 

The  treatment  necessarily  varies  according  to  the  precise  con- 
dition that  exists,  and  the  causes  on  which  it  depends.  The  removal 
of  these  as  far  as  possible,  and  of  any  influence  likely  to  depress  the 
nervous  system,  must  be  the  first  consideration.  It  should  be  remem- 
bered that  no  part  of  the  nervous  system  is  unaffected  by  mental 
depression,  and  that  impairment  of  the  general  health  may  render  im- 
possible recovery  from  local  disease.  From  these  general  principles 
the  details  of  the  treatment  of  individual  cases,  too  vaiious  to  be  here 
described,  may  readily  be  deduced.  Therapeutic  measures  that  are 
thus  reasoned  out  and  determined  on  by  the  indications  of  the  special 
case  are  far  more  likely  to  be  successful  than  those  that  are  simply  taken 
from  a  description  of  treatment.  The  only  points  that  need  special 
mention  are,  first,  the  importance  of  securing  mental  tranquillity  by 
producing  the  conviction,  when  possible,  that  no  grave  disease  exists 
or  is  impending,  and  with  it  the  disregard  of  those  slighter  sensations 
of  discomfort  that  become  more  aggressive  and  disabliag  the  more  they 
are  noticed.  At  the  same  time  it  is  important  that  the  patient  should 
avoid  aU  over-exertion,  all  risk  of  falls  or  chills,  and  should  be  espe- 
cially careful  in  regard  to  sexual  intercourse,  which  often  has  a  pecToliar 
effect  in  augmentiag  the  symptoms ;  and  in  the  unmarried,  perfect 
continence  should  be  enjoined.  Often,  indeed,  in  the  case  of  men,  the 
mental  preoccupation  causes  an  apparent  failure  of  sexual  desire,  which 
itself  is  a  source  of  concern — generally  groundless.  Reassurance  on 
this  poiat  frequently  helps  to  secure  the  desii-ed  mental  state.  Equally 
important  is  it  to  disabuse  the  sufferer's  mind  of  the  idea  that  seminal 
loss  is  weakening — an  error  widely  prevalent ;  the  truth  being  that  any 
depressiug  influence  comes  only  from  the  associated  nervous  action.  But 
it  is  important  to  lessen  undue  frequency  of  nocturnal  emissions,  and 
for  this  nothing  is  so  effective  as  Milton's  remedy,  large  doses  of  the 


APPENDIX,  671 

tinct.  ferri  percUor.  (^\^s.x  or  xxx)  tliree  times  a  day,  couiiled  witt 
the  avoidance  of  lying  on  the  back. 

The  relief  of  disabling  pain,  by  the  measTires  described  in  the 
chapter  on  neuralgia,  and  by  the  treatment  of  any  local  morbid  state, 
is  indispensable.  Eest  from  -work,  the  improvement  of  the  general 
health,  and  the  administration  of  nervine  tonics,  such  as  arsenic, 
phosphorus,  quinine,  strychnia,  and  the  like,  are  the  chief  other  points 
in  treatment.  A  course  of  massage,  or  of  gentle  faradisation  of  the 
muscles,  is  also  often  useful. 


APPENDIX.* 
THE  MUSCLE-SPINDLE. 

In  1862  Kolliker  first  described  the  muscle- spindle,  and  in  the 
following  year  Kuhne  added  considerably  to  the  description  given  by 
Holliker.  Both  these  investigators  considered  that  the  muscle- spindle 
was  a  stage  in  the  development  of  muscle  and  nerve,  and  although  in 
the  following  years  a  considerable  number  of  investigators  on  the 
Bubject  published  their  researches,  it  was  not  till  1888  that  Kerschner 
argued  the  sensory  nature  of  the  muscle- spindle.  In  1893  Euffini 
described  an  annulo-spiral  nerve  termination  within  the  spindle,  but  it 
remained  for  Sherrington  in  1894  to  prove  by  experiment  that  the 
nerve  supplying  the  spindle  passed  up  in  the  posterior  root  and 
definitely  to  settle  the  sensory  nature  of  the  muscle-spindle. 

Distribution. — Muscle- spindles  have  been  found  in  nearly  all 
skeletal  muscles  of  the  body,  excepting  the  eye  muscles  and  the 
diaphragm.  They  are  abundant  in  the  small  muscles  of  the  hand  and 
in  the  arm  muscles,  and  are  more  numerous  in  the  belly  of  the  muscle 
than  near  the  tendon.  In  relation  to  this  statement  it  is  well  to 
recognise  that  the  musculo-tendon  organs  are  foimd  to  be  numerous 
in  the  region  of  the  tendon.  The  muscle-spindle  is  present  at  all  ages 
from  the  fourth  month  of  fcetal  life  onwards. 

Size. — The  size  of  the  muscle-spindle  varies  considerably,  not  only 
in  the  same  muscle  but  also  with  regard  to  the  length  of  the  muscle, 
and  the  age  of  the  subject,  being  longer  in  the  adult  than  in  the  child, 

*  The  following-  account  of  the  muscle-spindle  has  been  kindly  written  by  Dr.  P. 
E.  Batten,  whose  able  and  industrious  researches  on  the  subject  render  this  ezprea* 
sion  of  Lia  views  most  valuable. 


672 


APPENDIX, 


and  in  the  larger  muscles.  Its  average  length  is  from  2  to  4  mm. 
(rg-  to  tV  of  ^^  inch),  but  it  may  measure  as  much  as  12  mm. 
(j  inch).  The  average  breadth  at  the  equatorial  region  is  from  '15  to 
•4  mm.  No  reliable  estimate  of  the  number  of  muscle-spindles  within 
the  muscles  has  yet  been  made,  but  seventy-nine  have  been  found  in 
ODe  biceps. 

Varieties  in  the  muscle- spindle  exist.  Thus,  they  are  not  always 
single,  but  may  be  compound,  by  the  junction  of  two  or  more,  so  that 
the  pole  of  one  spindle  enters  into  the  equatorial  region  of  another. 
Again,  it  is  not  uncommon  to  find  spindles  joined  at  their  poles ;  as 
many  as  three  have  been  found  in  a  row.  Eufl^i  distinguishes  three 
classes  of  spindles  according  to  the  complexity  of  their  nerve  termina- 
tions. 

The  muscle-spindle  lies  parallel  to  the  muscle-fibre  of  the  muscles  in 
which  it  exists,  and  not  unfrequently  parallel  to  a  nerve.  It  may  lie 
wholly  in  muscle  tissue,  or  partly  in  muscle  tissue  and  partly  in  the 
connective  tissue  round  the  muscle  bundles,  or  whoUy  in  the  connec- 
tive tissue. 

Description. — The  nomenclature  adopted  in  the  following  description 
is  that  suggested  by  Sherrington,  and  the  diagrams  given  will  show 
the  parts  alluded  to.  The  muscle-spindle  is  of  the  shape  which  its 
name  implies.    It  is  formed  bj  a  capsule  enclosing  two  or  more  fine 

Fia.  191. 

MliS.CLE    SpwdIH 


EOUATORIAI.   RecioH 


Pole  oasnn^ 


(ANNULOrgPtRftU^ 


IkRitajtyrfrowij 

PCSTEENOINS  f  ^f«■  KOTOS.') 


mnscle-fibres.  The  capsule  resembles  the  Henle  sheath  of  a  nerve,  and 
at  the  equatorial  region  of  the  spindle  consists  of  eight  or  more  laminae, 
while  at  the  poles  it  diminishes  to  a  single  lamina,  and  is  lost  on  the 
flheath  of  the  muscle-fibre. 

The  muscle-fibres  which  enter  the  spindle  are  of  smaller  size  than 
the  normal  fibres  composing  the  muscle,  the  intra-fusal  fibres 
measuring  about  '02  mm.,  while  the  extra-fusal  fibres  measure  about 
•06  mm.  As  a  rule,  two  or  three  of  these  fine  fibres  enter  the  pole  of 
a  spindle ;  as  they  pass  towards  the  equatorial  region  they  undergo 
division,  so  that  at  this  region  of  the  spindle  there  may  be  eight  or  ten 


APPENDIX. 


673 


fine  fibres,  some  of  ttem  measnriiig  only  '008  mm.  At  a  certain  point 
in  the  equatorial  region  some  of  the  muscle-fibres  lose  their  trans- 
verse striation  and  nuclei  appear  in  the  substance  of  the  muscle- fibre. 
These  nuclei  gradually  increase  in  number  till  they  completely  fill  the 
fibre,  then  after  a  short  distance  they  become  less  numerous,  and  the 
muscle-fibre  again  resumes  its  striation.  At  the  equatorial  region  the 
muscle-fibres  do  not  fill  the  whole  spindle,  but  lie  to  one  side.  As 
they  pass  to  the  distal  end  of  the  spindle  they  become  joiaed  again 
and  pass  out  of  the  spindle  as  two  or  three  fibres.  It  will  be  seen 
from  the  above  description  that  a  transverse  section  of  the  spindle 
may  at  a  given  point  in  the  equatorial  region  seem  to  contain  no 


Pio.  192. — Normal  spindle  of  child  in  transverse  section.    Magnified 
260  diameters. 

1.  Section  near  the  pole.    The   areas  marked  with  veitical  lines  are 
muscle-fibres  in  transverse  section. 

2.  Nerve  entering  into  spindle.     Many  of  the  dark  bodies  in  contact 
with  the  muscle-tibres  are  nerve-fibres. 

3.  Nearer  the  equatorial  region  the  sheath  has  acquired  considerable 
thickness. 

4.  At  the  equatorial  region  the  muscle-fibres  have  undergone  consider* 
able  modification,  and  are  no  longer  recognisable  as  such. 

6.  The  spindle  near  the  opposite  pole ;  the  muscle-fibres  have  resumed 
their  natnral  appearance. 

6.  At  the  distal  pole  of  the  spindle. 

VOL.  I.  43 


674  APPENDIX. 

nmscle-fibres,  tlie  structures  of  such  having  been  so  profoundly 
modified  (Fig.  192,  4). 

Nerve-supply. — The  nerve-supply  of  the  spindle  is  always  large — as 
a  rule  at  least  two  nerves  pass  to  it,  one  at  the  equatorial  region  and 
another  at  the  distal  or  proximal  end.  It  is,  however,  by  no  means 
uncommon  to  find  spindles  with  four  or  five  nerves.  The  nerve  passing 
to  the  equatorial  region  always  contains  a  nerve-fibre  of  large  size 
measuring  about  '008  mm.  This  nerve  on  entering  the  spindle  loses 
first  its  Henle  sheath,  which  fuses  with  the  sheath  of  the  spindle,  and 
next,  after  entering  its  medullary  sheath,  it  then  divides  into  two  and 
forms  the  annulo- spiral  nerve  termination  of  Euffini,  winding  round 
the  muscle-fibres  at  the  point  where  the  nuclei  are  situated  in  the 
muscle-fibre.  From  the  nerve  that  enters  the  equatorial  region  other 
fibres  pass  towards  the  poles  of  the  spindle  and  lie  between  its  muscle- 
fibres  ;  the  mode  of  termination  of  these  fibres  is  imcertain  in  man,  but 
probably  corresponds  to  the  secondary  ending  described  by  Euffini. 

The  nerves  which  enter  the  poles  of  the  spindle  are  nearly  always 
composed  of  fine  nerve-fibres  having  a  diameter  of  '004  mm. ;  they 
likewise  pass  between  the  muscle-fibres  of  the  spiadle  and  pass  towards 
the  equatorial  region. 

The  question  whether  the  muscle-spindle  contains  a  motor  nerve 
termination  is  one  that  has  not  yet  been  determined,  for  the  third 
form  of  nerve  ending  described  by  Euffini  and  named  the  "  plate 
ending"  is  said  by  him  not  to  be  motor.  Owing  to  the  number  of 
nei"ve-fibres  within  the  spindle  there  is  a  plexiform  arrangement  of 
nerves  between  the  muscle-fibres  and  the  sheath  of  the  spindle,  but  so 
far  as  can  be  ascertained  they  do  not  intercommunicate  to  form  a  true 
plexus.     These  nerves  are  myehnated. 

Blood-vessels. — The  muscle-spindle  is  supplied  by  arteries  and  veins, 
which  most  frequently  enter  the  spindle  near  its  equatorial  region. 

TJnd&i'  certain  pathological  conditions  the  muscle-spindle,  owing  to 
its  apparent  immunity  from  change,  stands  out  in  striking  contrast  to 
the  surroundiag  muscle  tissue.  In  all  wasting  diseases,  and  especially 
in  phthisis,  the  muscle-spindle  forms  a  very  marked  feature  in  sections 
of  the  muscle,  but  no  change  has  been  demonstrated  in  the  structure 
of  the  spindle.  It  is  for  the  same  reason  more  striking  in  the  muscles 
of  a  child  than  those  of  an  adult. 

In  infantile  paralysis,  although  all  the  surrounding  muscle  tissue 
may  have  been  atrophied  or  have  undergone  fatty  degeneration,  yet 
the  muscle-spindle  is  normally  preserved  and  supphed  by  a  perfectly 
normal  nerve. 

Similarly  in  progressive  muscular  atrophy  exactly  the  same  condi- 
tion can  be  demonstrated,  although  in  these  cases  the  atrophy  of  the 
surroundiag  muscle  may  not  be  so  extensive. 

In  the  myopathies  the  muscle- spindle  forms  a  very  striking  appear- 
ance, as  is  shown  by  the  description  given  of  them  in  pubHshed  cases 
of  this  disease.    They  remain,  however,  perfectly  normal  both  with 


APPENDIX.  675 

regard  to  their  nerve-supply  and  the  character  of  their  muscle- 
fibres. 

In  peripheral  neuritie  certain  changes  have  been  described  within 
tli:^  mnscle-spindle  both  as  regards  the  muscle- fibres  and  also  as 
regards  the  nerves;  in  other  cases,  however,  they  have  been  shown 
to  be  perfectly  normal. 

In  tabes  dorsalia,  although  the  external  form  of  the  spindle  and  the 
nerves  passing  to  it  have  been  found  to  be  normal,  certain  changes 
have  been  described  in  the  intra-fusal  muscle-fibres.  The  evidence, 
however,  on  this  point  is  insufficient  at  the  present  time  to  justify  us 
in  regarding  the  changes  described  as  constant  in  this  disease. 

Beferencea. — Shkbbington,  'Journal  of  Physiology,'  voL  xvii,  p.  237,  1895 1 
Ru?7iNi,  'Joarnal  of  Physiology/  toL  zziU*  p,  190^  1898}  Bahbn,  'Biain,' 
toLzz,p.138,1897. 


INDEX. 


A 


Abdominal  reflex,  20 
Abductor  minimi  digiti,  62 

—  pollicis,  44,  52 
Abscess  of  spinal  cord,  377 
Accessory  hsematoinyelia,  488 
Action,  antergic,  10 

—  reflex,  19 

—  synergic,  of  muscles,  10 
Acute  anterior  polio-myelitis,  396 

—  ascending  paralysis,  371,  427 

—  atropbic  paralysis,  396 

—  myelitis,  344 
Adductor  brevis,  47 

—  longus,  47 

—  magnus,  47 

—  poUicis,  52 
Adductors  of  tbigh,  47»  232 
^sthesiometer,  14 
Afferent  tract  crossed,  223 

Albinism  and  pseudo-bypertropbio  para- 
lysis, 569 

Alcoholic  ataxia,  168,  159  {see  Pseudo- 
tabes) 

—  meningitis,  chronic,  824 

—  neuritis,  150 

—  pseud  o-tabes,  159, 173, 175,  372,  481 
Alcoholism  in  myelitis,  347,  380 
Alimentary  canal,  nerve-centres  for,  247 
AUocbeiria,  14,  453 

—  in  tabes,  453 
Amyloid  bodie>,  ;U8,  527 
Amyotrophic    liiunil    sclerosis,  581    {see 

Progressive  muscular  atrophy) 
Anemia  of  spinal  cord,  839,  340,  513 
Anaesthesia,  13 

—  dolorosa,  260,  393 
Analgesia,  15 
Analgic  panai-itinm,  645 
Anastomotic  artery,  227 

Anatomical  diagnosis  in  cord  disease,  253 


Anatomy  of  spinal  cord,  200 

Aneurism,  erosion  of  vertebrae  by,  305 

Angioglioma,  609 

Angiosarcoma,  609 

Angular  curvature,  287,  290,  291 

Ankle-clonus,  22 

Annular  myelitis,  chronic,  882 

—  sclerosis,  382 
Anode,  73 

Antergic  contraction,  10 
Anterior  central  artery,  227 

—  column,  201 

—  cornua,  anatomy  of,  204 

—  crural  nerve,  diseases  of,  115 
— neuritis  of,  134 

—  ground-fibres,  223 

—  lateral  arteries,  227 

—  median  arteries,  226 

—  — •  fissure,  201 

—  polio-myelitis,  395 

—  pyramidal  tracts,  207,  209 

—  radicular  arteries,  227 

—  root-fibres,  207,  224 

—  spinal  artery,  226 

vein,  227 

Antero-lateral  column,  201 

—  tract,  ascending,  201,  208,  222,  470 

—  —  degeneration  in  tabes,  470 

—  —  descending,  215,  223 
Arachnitis,  809 

Argyll  Kobertson  pupil,  455 

Arm,  combined  palsies  of  nerves  of, 

•—  diseases  of  nerves  of,  110 

—  motor  points,  38,  39 

—  muscles,  84 

Arteries  of  spinal  cord,  226 — 228 
Arthritic  muscular  atrophy,  559 
Arthritis,  vertebral,  307 
Articulations  of  spine,  diseases  of,  807 
Ascending  neuritis,  82,  85 

—  paralysis,  acute,  371,  427 
Ataxic  paraplegia,  503 — 511 


678 


INDEX. 


Ataxy,  258 

—  cerebellar  hereditary,  524 

—  hereditary  (Friedreich),  519 

—  in  multiple    neuritis,   153,  159    (see 

Pseudo-tabes) 

—  in  tabes,  449 

—  of  movement,  10 
Athetosis,  double,  496 

Atrophic  spinal  paralysis,  acute,  396 

—  subacute  and  chronic,  424 

Atrophy,  atonic  muscular,  540 

—  idiopathic  muscular,  564 

—  muscular,  from  over-use,  563 

family  form  of,  with  spinal  lesion, 

597 
peroneal  form,  593 

—  of  auditory  nerve,  457 

—  of  optic  nerve,  457,  503 

—  progressive  muscular,  531 

—  pseudo-hypertrophic,  564,  567 
Axis-cylinder,  compound  character  of,  55, 

62 

—  process,  65 
Axites,  56 
Axon,  55,  204 

B 

Bedsores,  changes  in  nerves  near,  68,  83 
Bending  of  spine,  violent,  586,  591 
Beri-beri,  186 
Biceps  cruris,  47 
—  muscle,  39 
Bladder,  centre  for,  245  (see  Sphincters) 

—  condition  of,  in  pseudo-hypertrophic 

paralysis,  575 

—  ulceration  of,  in  myelitis,  362 
Blood-supply  of  cord,  226 
Bones  in  infantile  palsy,  404i 

—  in  syringomyelia,  643 

—  nutrition  of,  245 
Brachial  neuritis,  119 

—  plexus,  diseases  of,  98 
Brachialis  anticug  musr>le,  83 
Bronchial  crises,  482 
Brown-Sequard,  experiments  oa  sensation, 

234 
Burdach's  column,  200, 219 


Caisson  disease,  433 
Calf  muscles,  centres  for,  232 
C^fUus,  effects  of,  on  nerves,  101 
Quput  cornu  posterioris,  206,  225 


Carcinoma  of  cord,  608 

Caries  of  spine  and  tumour,  803 

—  —  disease  of  cord  in,  286 
syphilitic,  305 

Cauda  equina,  tumour  of,  216,  610 

—  and  syringomyelia,  636 

Cautery,  actual,  in  traumatic  lesions,  664 
Cavities  in  cord,  629 
Cell-body,  structure  of,  59 
Cells  of  anterior  horn,  204 

—  unipolar,  57 
Cellulif  ugal  dendrites,  58 
CelluHpetal  dendrites,  58 
Central  canal,  224 
Cerebellar  ataxy,  hereditary,  524 

—  tract,  direct  or  dorsal,  207,  209,  220 
ventral,  208, 222  («e«  An  tero-lateral 

ascending) 

—  tumour  and  tabes,  483 
Cerebro-spinal  motor  segment,  213 
Cervical  enlargement,  localisation  in,  231 

—  muscles,  centres  for,  231 

—  vertebrae,  fracture  of,  283 
Charcot's  joint  disease,  266,  459,  643,  652 
Cheyne-Stokes  breathing,  318 

Chronic  atrophic  paralysis,  531 

—  meningitis,  322 

—  myelitis,  378 

—  polio-myelitis,  424 

—  spinal  muscular  atrophy,  5S1 
Circulation  in  cord,  226 
Circumflex  nerve,  disease  of,  103 
Clarke's  column,  206,  221 

•—  —  in  tabes,  471 
Clasp-knife  rigidity,  265,  493 
Classification  of  nervous  diseases,  1,  8 
Claw-like  hand,  43, 109,  639 
Clonus,  foot,  11,  12,  22 

—  spurious,  501 
Coarse  disease,  1 

Cold,  exposure  to,  in  cord  disease,  277 
Collaterals,  55 

Column,    antero-lateral   ascending,    201, 
208,  222,  470 

—  —  descending,  215,  223 

—  of  Burdach,  200,  219 

—  of  Clarke,  206,  221 

—  of  GoU,  200 

—  of  Gowers,  201,  208,  222,  470  (««• 

Antero-lateral  ascending) 

—  of  Tiirck,  210 

—  posterior,  215 


INDEX. 


679 


Column,  posterior  vesicular,  206 

—  postero-external,  200,  219 

—  postero-median,  200, 207 
Combination  of  symptoms  in  cord  disease, 

269 
Comma-shaped  degeneration,  216,  219 
Commissural  artery,  226 
Commissures  of  cord,  201,  223,  224 
Compression,  effects  of,  on  nerves,  80 

—  myelitis,  276 

—  of  cord,  390 
Concussion  myelitis,  652 
Conduction  in  cord,  228,  233 
Congenital  paramyotone,  605 

—  spastic  paraplegia,  495 

—  tabes  and  spina  bifida,  651 
Congestion,  2 

Contraction,  fibrillary,  8,  257,  539 

—  idio-muscular,  8 

—  in  pseudo-hypertrophic  paralysis,  574 

—  myotatic,  26 
Contracture,  12,  256 

—  after  facial  palsy,  77 
Contra-lateral  muscles,  84 
Convulsions  in  cord  disease,  269 
Co-ordination  of  movement,  11,  242 
Cord,  274 

—  abscess  of,  377 

—  affection,  symptoms  of,  253 

—  anatomy,  199 

•—  blood-supply  of,  226 

—  cavities  and  fissures  in,  629 

—  degenerations  of,  442 

—  diseases  of,  199 

—  embolism  of,  377 

—  functions,  228 

■^  grey  substance  of,  203 

—  in  peripheral  neuritis,  168,  l76 

—  inflammation  of,  343 

—  membranes  of,  199 

—  softening  of,  275,  276 

—  tumours  of,  607 
Cornu-commissural  tract,  220 
Corpora  amylacea,  348,  527 
Cramp,  17 

Cremasteric  reflex,  20,  232 
Crises  in  tabes,  462 
Crossed  afferent  tract,  223 

—  pyramidal  tracts,  209 
•—  sensation,  272 
Cross-legged  progression,  496 
Croreus  muscle,  47 


Crutch  palsy,  105 

Cuiieate  nucleus,  219 

Curvature,  angular,  287,  290,  291 

—  of  spine,  lateral,  307 

—  in  Friedreich's  disease,  523 

7—  in  idiopathic  muscular  atrophy,  688 

—  in  infantile  paralysis,  408 

—  in  pseudo-hypertrophic  paralysis,  575 

—  in  progressive  muscular  atrophy,  538 

—  in  syringomyelia,  642 

—  lateral,  307 
Cutaneous  reflexes,  20 

—  sensibility,  representation  of,  in  cord, 

238 
Cysticerci  of  cord,  609 
Cysts  in  cord  tumours,  614 

D 

Deafness  in  tabes,  457 

Decussation  of  sensory  fibres  in  cord,  234^ 

235 
Deep  reflexes,  19,  20,  238 
Deformities  from  infantile  palsy,  405 
Degeneration,  4 

—  bilateral,  in  cord,  211 

—  comma-shaped,'  of  cord,  216,  219 

—  fatty,  of  muscle,  546 

—  granular,  546 

—  in  cord,  217 

—  in  myelitis,  355 

—  irritative  character  of,  68 

—  reaction  of,  in  muscle,  31,  32 
in  sensory  nerves,  405 

—  retrograde,  215  (foot-note) 

—  secondary,  of  nerves,  65 

—  Wallerian,  65 
Degenerations  of  cord,  274 

ascending,  208,  215 

descending,  208,  210 

—  vitreous,  of  muscle,  70,  546 
Degenerative  neuritis,  32 

—  reaction,  32 
Deiter's  cells,  201,  351 

Delay  of  sensation  in  tabes,  452 
Deltoid  muscle  and  centre,  36,  231 
Dendrite,  55 

—  celluli  petal,  58 
Dendron,  55 

Descending  antero-lateral  tract,  216 

—  degenerations,  208,  210,  223 
Deuteropathic       progressive       muscular 

atrophy,  532 


630 


INDEX. 


Development  of  cord,  629 
Diabetes,  sclerosis  in,  513 
Diagnosis,  pathological,  274 
Diaphragm,  action  of,  33 

—  centre  for,  231 

—  inflammation  of,  97 

—  paralysis  of,  33,  99,  537 
DilVuse  niyelitis,  344 
Diphtheria  and  cord  disease,  278 
Diphtheritic  neuritis  and  tabes,  483 
Diiect  cerebellar  tract,  207,  209,  220 

—  pyramidal  tract,  207,  209 
Disease,  nature  of,  278 

—  nutritional,  2,  664,  668 

—  organic,  2 

—  seat  of  indications  of,  278 

—  structural,  2 
Dislocation  of  spine,  281 
Disseminated  myelitis,  351,  353,  865,  370 

—  neuritis,  84 

—  sclerosis,  275 
Diver's  paralysis,  453 
Dorsal  reflex,  20 

—  vertebrae,  fracture  of,  286 
Duchenne's  disease,  445 

Dura  mater,  haematoma  of,  323,  327 

tumours  of,  308,  607 

Dynamometer,  9 
Dyssesthesia,  15 
Dyslexia,  341 

Dystropliies,  muscular,  563 
Dystrophy,  muscular,  563 

E 

Echinocoeci,  543 

Elbow  extensors,  centre  for,  231 

—  flexors,  centre  for,  231 
Electrical  irritability,  29 — 31 

—  —  in  functional  disease,  32 

—  reaction,  changes  in,  75 

in    progressive  muscular  atrophy, 

539 

—  treatment  in  infantile  paralysis,  422 
Elephantiasis  neuromatodes,  93 
Embolism  of  cord,  377 

Embryonal  tissue  and  syringomyelia,  629, 

632 

and  tumours,  613,  634 

in  nerves  in  Friedreich's  disease, 

629 

—  —  persistence  of,  biJy 


Emphysema,  subcutaneous,   in    myelitiflt 

362 
Empyema,  paralysis  from,  665 
Encephalomyelitis,  disseminated,  344^  365 
Enchondroma  of  cord,  608 
Endemic  neuritis,  185 
Epidemic  meningitis,  cerebro^spinal,  822 

partial,  320 

Epigastric  reflex,  20 

Erb's  paralysis,  112,  407,  565 

Erosion  of  spine  by  aneurism,  305 

Essential  paralysis  of  children,  895  (set 

Infantile  paralysis) 
Excentric  pain,  260 
Excitation,  61 
Exophthalmos    in    pseado  •  hypertrophie 

paralysis,  587 
Exostoses,  vertebral,  304 
Exposure  in  cord  disease,  277 
Extensor  quadriceps,  47 
Extensors,  nerve-centres  for,  232 

—  of  ankle,  50, 52 

—  of  elbow,  231 

—  of  fingers,  40,  232 

—  of  foot,  51 

—  of  knee,  47,  232 

—  of  thumb,  42—44 

—  of  toes,  50,  52 

—  of  wrist,  40,  231 
External  meningitis,  309 

—  ophthalmoplegia,  457 

—  popliteal  nerve,  disease  of,  117 
Extra-dural  tumours  of  cord,  607 
Extra-meningeal  hsemorrhage,  320 
Eye  symptoms  in  tabes,  457 
Eyeballs,  trophic  changes  in,  28 


Pace  in  idiopathic  muscular  atrophy,  587 

—  in  neuritis,  161 

—  in  progressive  muscular  atrophy,  538 
Facial  nerves  in  leprous  neuritis,  193 

—  type  of  muscular  atrophy,  665,  587 
False  neuroma,  90 

Paradism,  29.  75 

—  in  infantile  palsy,  422 
Fasciculi  of  nerve-fibres,  63 
Patty  degeneration  of  muscle,  646 
Fibres,  grey,  63 

—  non-meduUated,  63 

—  regreneration  of,  in  cord,  356 


INDEX. 


681 


Pibrilbtion  of  muscle,  8,  257,  539 
Fibrils,  cellulif  iigal,  58 

—  primitive,  55,  56,  62 
Fibromata  in  nerves,  90 

Fifth   nerve,  degeneration  of,  in  tabes, 

451,  453,  474 
Fingers,  extensors  of,  40,  232 

—  flexors  of,  41,  232 
Fissures  in  cord,  629 
Flat-foot,  50 
Fiexibilitas  cerea,  27 
Flexors,  nerve-centre  for,  232 

—  of  elbow,  231 

—  of  fingers,  41,  232 

—  of  foot,  52 

—  of  knee,  232 

—  of  wrist,  40,  232 

—  long,  of  fingers,  52 

—  short,  of  fingers,  52 

—  long,  of  thumb,  44 
■ —  short,  of  tliunab,  44 

of  little  toe,  52 

Foot  muscles,  48.  232 

—  —  nerve- centres  for,  232 

—  phenomenon,  22 
Foot-clonus,  12,  22 

—  nerve-centres  for,  232 
Forearm,  muscles  moving  the,  87 
Fracture  of  spine,  282 
Fractures,  spontaneous,  of  bone,  459 
Friedreich's  disease,  519 
Functional  disease,  1,  276,  664 
Functions,  mutual  relations  of,  249 
<i—  of  cord,  228 

G 

Gait  in  Friedreich's  ataxy,  520 

—  in  pseudo-hypertrophy,  669,  735 

—  in  spastic  paraplegia,  494 

—  in  tabes,  449 
Ganglion-cells,  203,  204 
Gastric  crises,  462 
Gastrocnemius,  48 
Gelatinous  grey  substance,  203 
Gemelli  muscles,  46 

General  causation  of  cord  disease,  277 

—  paralysis  and  ataxic  paraplegia,  507 

and  tabes,  446,  466,  482 

Girdle-pain,  261,  360, 657 
Glia-cells,  201 

Glioma  in  nerves,  91 

—  of  cord,  610 


Gliomatft  in  relation  to  nervout  system,  4 
Gliomatosis,  4,  632 
Glossy  skin,  28,  86 
Gluteal  muscles,  45,  232 

—  reflex,  20 

Gluteus  maximus,  45,  232 

—  medius,  45,  232 

—  minimus,  46,  232 
Glycosuria  in  tabes,  467 

Golgi's  method,  effect  of,  on  conceptions 

of  nervous  system,  53 
Goll's  column,  200 
Gonorrhoea  and  myelitis,  278,  347 

—  and  neuritis,  147 

Gout  and  cord  disease,  380,  660 

—  and  myelitis,  380 

—  and  neuritis,  137 

Gowers'   tract,   201,   208,   222,  470   (tee 

Antero-lnteral  ascending) 
Gracilis  muscle,  47 

Granular  degeneration  of  muscles,  546 
Grey  commissure,  201,  224 

—  substance,  203 

of  Rolando,  206 

Ground-fibres,  223 

Growths  in  spinal  column,  299 

cord,  299,  607 

Gunshot  wounds  of  spine,  652 


Hsematoma  of  dura  mater,  323,  327 
Hsematomyelia,  437 
Haematorrhachis,  334 
Haemorrhage  into  cavities  of  cord,  438^ 
644 

—  into  central  canal,  653 

—  into  cord,  320,  437 

—  into  spinal  membranes,  320,  834 

—  —  —  —  and  meningitis,  320 
Hsemorrhagic  myelitis,  348,  359,  366 
Hand  in  idiopathic  muscular  atrophy,  566 

—  in  progressive  muscular  atrophy,  539 

—  in  pseudo-hypertrophic  paralysis,  571 

—  muscles  moving  the,  40 

—  —  nerve-centres  for,  232 

Heart  action  in  cervical  cord  disease  267 
in  tabes,  267 

—  disease  in  tabes,  466 
Hemiatrophy  of  tongue,  409 
Hemiparaplegia,  254,  272 
Hemiplegia,  spinal,  272 
Henle's  gheath,  P^ 


682 


INDEX. 


Hereditary  ataxy,  519 

—  cerebellar  ataxy,  524 
Herpes  zoster  in  caries,  290 
Herpetic  neuritis,  196 
Hip-joint,  muscles  moving  the,  45 

—  nerve-centres  for,  232 
Horn,  lateral,  203,  205 

Humerus,  dislocation  of,  and  paralysis.  Ill 

—  fracture  of,  and  paralysis,  IH 
Hutchinson's  mask,  453 
Hyaloplasm,  56,  60 

Hydatid  disease  of  cord,  609 

of  spine,  306 

Hydromyelia,  628 
Hydrorrhachis  interna,  628 
Hypersemia  of  cord,  339,  342 
Hyperaesthesia,  15,  259 

—  in  cord  disease,  259 
Hypei'algesia,  15,  259 

—  in  cord  disease,  259 
Hyperpyrexia  in  cord  disease,  268 
Hypertrophic  pachymeningitis,  326,  623 
Hypertrophy,  muscular,  599 
Hypotonic  musculaire,  256 

in  tabes,  461 

Hysteria  and  myelitis,  372 
Hysterical  paraplegia,  665 


Idio-muscular  contraction,  8 
Idiopathic  muscular  atrophy,  583 

—  neuritis,  146 
Diacus  muscle,  46,  232 
Impulses,  nervous,  origin  of,  58 
Incisions  of  Schmidt,  63 
Incontinence  of  urine,  246,  361 

—  —  overflow,  246,  361 

reflex,  246,  361 

Inco-ordination,  meciianism  of,  477 

—  of  movement,  10,  257 
Indentations  of  Lautermann,  63 
Infantile  paralystp,  396 

—  —  regression  in,  408 
Infection  in  cord  diseases,  277 
Inflammation  of  cord,  343 

—  of  membranes,  322 

—  of  nerves,  82 

—  parenchymatous,  4 
jnfra-spinatus  muscle,  37 

in  pseudo-hypertrophic  palsy,  570 

Injury  in  cord  disease,  277 
^  to  nerves,  80 


Intercostals      in     progressive      muscular 

atrophy,  537 
Intermediate  grey  substance,  203 

—  group  of  cells,  205 

—  process,  205 

—  septal  artery,  227 

—  septum  of  cord,  215 
Intermedio-lateral  tract,  205 
Internal  meningitis,  313 
chronic,  322 

—  popliteal  nerve,  117 
Internode,  62 
Interossei  of  foot,  51,  52 

—  of  hand,  41 

Interstitial  neuritis,  82,  83,85 
Intra-meningeal  hsemorrhage,  320 
Intra-spinal  tumours,  607 
Irritability,  electrical,  of  nerve  and  muscle^ 

29,71 

—  of  muscle,  reflex,  26 
Irritation,  spinal,  660 

Irritative  character  of  secondary  degenera» 
tion,  68 

—  degeneration  of  pyramidal  fibres,  266 


Jaw-jerk,  495 
Jendrassik's  method,  21 
Joints  in  infantile  palsy,  405 

—  in  tabes,  459 

—  nutrition  of,  245 
Juvenile  muscular  atrophy,  665 

K 

Kak-k^,  186 
Kathode,  73 
Kidney  disease  in  ataxic  paraplegia,  466 

in  tabes,  416 

Kinetoplasm,  60 
Klumpke's  paralysis,  113 
Knee,  extensors  of,  47,  232 

—  flexors  of,  47,  232 

—  muscles  moving  the  knee,  47 
Knee-jerk,  20,  21,  24 

—  centre  for,  239,  252 

—  in  diphtheria,  477  (footnote),  481 

—  in  Friedreich's  disease,  521 

—  in  idiopathic  muscular  atrophy,  588 

—  in  leprous  neuritis,  198 

—  in  pseudo-hypertrophic  palsy,  575 

—  in  tabes,  448 

—  latent  period  of,  25 


INDEX. 


68S 


Kne©-jerk,  loss  of,  264,  272,  448,  617 

—  retarn  of,  in  tabes,  455  (footnote),  464 

Kolliker,  63 


Laceration  of  cord,  652,  654 

Lameness,  intermittent,  341 
Landry's  paralysis,  371,  427 
.—  —  and  neuritis,  427 
Laryngeal  crises,  462 

—  nerves,  affections  of,  in  neuritis*  161 

—  —  —  in  tabes,  462 
Latent  period,  24 

Lateral  arteries  of  cord,  227 

—  column  of  cord,  201 

^  curvature,  symptoms  of,  307  (see  Lor- 
dosis) 

—  horn,  205 

—  limiting  layer,  207,  210,  221 
^  sclerosis,  primary,  489 

—  tracts,  207,  209,  492 
Lathyrisin,  37 

Latissimus  dorsi    in  pseudo-hypertrophy, 

580 
Lautermauji,  indentations  of,  63 
Lead  palsy,  distribution  in,  161 
Leg,  centre  for,  232 

—  disease  of  nerves  of,  114 

—  motor  points  of,  46,  48,  49 

—  muscles  of,  45 
Leprosy,  anesthetic,  192,  194 

mutilations  in,  195 

Leprous  neuritis,  192 
Leptomeningitis,  309 

—  chronic,  323 
Leucocythsemia,  nerves  in,  85 
Levator  anguU  sc^ipiilse,  35 
Lightning  pains,  448,  451 
Limb,  lower,  nerves  of,  14 

—  —  paralysis  of,  114 

—  ujjper,  nerves  of,  98 

paralysis  of,  100 

Lipoma  of  coid,  608 
Lipomatous  muscular  atrophy,  567 
Lissauer's  tract,  221,  225,  226,  469 
Live  flesh,  9 

Localisation  of  function  in  cord,  230 
Locomotor  ataxy  (see  Tabes),  444 
Lordosis  in  Friedreich's  ili^ease,  523 

—  in  idiopathic  muscular  atrophy,  588 

—  in  progressive  muscular  atrophy,  538 


Lordosis  in  pseudo-hypertrophic  muscular 

atrophy,  575 
Lower  limbs,  muscles  of,  45 
Lukewarmness,  16 
Lumbar  enlargement,  localisation  in,  232 

—  plexus,  diseases  of,  115 

—  region,  tumours  of,  620 
Lumbricales  of  foot,  52 

—  of  hand,  41 

M 

Main-en-grifEe,  43,  109,  539 

Median  nerve,  disease  of,  107 

Medullary  sheath,  62 

Membranes  of  cord,  diseases  of,  199,  308 

tumours  of,  607,  609,  615,  619 

Meningeal    hsemorrhage   and   meningitis^ 

320 
Meningitic  streak,  318,  618 
Meningitis  and  hajmatomyelia,  320 

—  and  primary  lateral  sclerosis,  323 

—  and  rheumatism,  320 

—  and  tetanus,  320 

—  spinal,  309 

—  —  external,  309 

—  —  internal,  313 

—  —  —  acute,  313 

— chronic,  322 

Meningocele,  650 
Meningo-myelitis,  313,  384 
Meningo-myelocele,  650 
Menstruation  and  myelitis,  346 
Mental  distress  in  cord  disease,  277 
Metameres,  199 

Micturition,  mechanism  of,  247 
Middle  form  of  reaction,  32,  393 
Mixed  reaction,  32,  393 
Morphia  in  neuritis,  89 
Morvan's  disease,  645 

—  —  and  Eaynaud's  disease,  647 

—  —  neuritis  in,  647 

—  —  sensibility  in,  646 
Motor  conduction  in  cord,  228 

—  over-action,  255 

—  path,  213,  228 

—  points  for  arm,  38,  39 
for  leg,  46,  48,  49 

—  segments,  disease  of,  254 
upper  and  lower,  213,  254 

—  symptoms,  8 

in  cord  disease,  254 

— •  —  in  tabes,  449 


€84 


INDEX. 


Movement,  co-ordination  of,  10,  242 

—  direction  of,  11 

—  inco-ordination  of,  10,  257 

—  spontaneous,  11,  450 
Movements,  representation  of,  in  cord,  230 
Multiple  neuritis,  138 
<—  neuroma,  92,  609 

—  tumours  of  cord,  621 

—  —  of  nerve-roots,  621 

— and  tabes,  621 

Muscle  spindles,  16,  671 
Muscle-reflex  action,  20 

—  centres,  240 

Muscles,  action  and  paralysis  of,  88 
r—  antergic,  action  of,  10 

—  changes  in,  29 

—  —  in  neuritis,  168         , 
^  —  secondary  to  nerve  lesion,  70 

—  contra-lateral,  34 

—  fatty  degeneration  of,  546 

—  in  peripheral  neuritis,  70, 168 

—  nutrition  of,  244 

—  nutritive  changes  in,  70 

—  peripheral  guidance  of,  11 

—  reflex  action,  20 

—  sensory  nerves  of,  17,  26 

—  synergic  action  of,  10 

—  vitreous,  70,  546 
Muscular  anaesthesia  in  tabes,  454 

—  atrophy,  arthritic,  559 
-^  —  chronic  spinal,  531 

f'Hcial  type,  565,  587 

^  —  family  form  in  children  with  spinal 

lesion,  597 
^  —  from  over-use,  563 

—  —  idiopathic,  583 
in  lead,  161,  534 

—  —  in  tabes,  466 

—  —  Landouzy-Dejerine  type,  565 

—  —  peroneal  type,  534,  593 

—  contraction,  paradoxical,  27 

—  dystrophies,  563 

—  exertion,  excessive,  and  cord  disease 

278 

—  hypertrophy,  578,  599 

—  sense,  16,  18,  237,  259 

—  sensibility,  16 

— test  for,  18 

Musculo-cutaneous  nerve,  104 
Musculo-spiral,  104 
Myelin,  62,  63 

Myelitis,  acute,  844 


Myelitis  and  alcoholism,  347 

—  and  cold,  345 

—  and  fevers,  346 

—  and  gonorrhoea,  347 

—  and  gout,  380,  660 

—  and  hysterical  paralysis,  372 

—  and  injury,  345 

— ■  and  Landry's  paralysis,  371 

—  and  meningitis,  320,  372 

—  and  menstruation,  345 

—  and  multiple  neuritis,  372 

—  and  muscular  action,  345 

—  and  organisms,  346,  347 

—  and  peripheral  irritation,  347 

—  and  smallpox,  347 

—  and  syphilis,  347,  380,  384 

—  and  toxic  blood-states,  346,  368 

—  central,  355,  366,  370 

—  chronic,  378 
annuhir,  383 

—  compression,  276,  353,  391 

—  diffuse,  344 

—  disseminated,  351,  353,  365,  370,  878 

—  focal,  365 

—  girdle-pain  in,  360 

—  hsemorrhagic,  348,  359,  366 

—  parenchymatous,  366 

—  reflexes  in,  360 

—  sclerotic,  863 

—  sphincters  in,  361 

—  ti  ansverse,  365,  378 

—  tiopliic  changes  in,  362 
Myolipoma  of  cord,  580,  608 
Myopathic  atrophy,  563 

face,  565,  587 

Myopathies,  Erb's  juvenile,  564 

—  idiopathic,  583 

—  Landouzy-Dejerine,  565 
Myosis,  spinal,  266,  455 
Myositis,  168,  185 
Myotatic  contractions,  26,  238 

—  irritability,  19,  238 

in  arthritic  atrophy,  560 

Myotonia  congenita,  601 
Myxo-fibroma,  91 
Myxoma  in  nerves,  90,  91 
Myxomata  of  cord,  609,  618 


N 


Nails  in  syringomyelia,  642 
Nansen's  researches,  53 


INDEX. 


68! 


"Seek,  Injuries  of,  and  paralysis,  111 
Nephralgic  crises,  462 
Nerve,  anterior  cruml,  115 

—  cells,  functions  of,  58 

—  —  relation  of,  to  nerves,  57 

—  circumflex,  103 

—  endings,  changes  in,  69 

—  energy,  65 

—  fibres,  functions  of,  64 
sizes  of,  207 

•^  force,  generation  of,  57 

—  impulse,  nature  of,  61 

—  inflammation,  82 

—  injury  and  degeneration,  71,  80 

—  lesions,  78,  79 

—  median,  107 

—  musculo-cutaneous,  104 

—  musculo-spiral,  104 

—  obturator,  116 
■^  phrenic,  96 

—  plantar,  117 

^  posterior  thoracic,  101 

—  poplitOiil,  117 
^  Bciatic,  116 

■^  superior  jrlnteal,  116 

—  Bupra-scapular,  102 

—  ulnar,  108 
Nerve-roots,  cervical,  99 

—  dorsal,  99 

—  lumbar,  114 
Nerve-stretching  in  tabes,  489 
Nerves,  bulbous,  93 — 95 

—  compression  of,  80 
— •  diseases  of,  62 

—  growths  in,  90 

—  inflammation  of,  82 

—  irritation  of,  in  caries,  288 

—  lesions  of,  65 

—  of  arm,  combined  palsies  of,  110 

—  of  lower  limb,  114 

—  of  upper  limb,  98 

—  physiology  of,  63 

—  regeneration  of,  66,  69 
■^  sclerosis  of,  84 

—  secondary  degeneration  of,  65,  66 

—  special,  diseases  of,  96 
Nervi  nervorum,  63 

Nervous    system,   diseases    primary 

secondary,  3 
.^  ^  function,  7 

—  —  general  constitution  of,  53 
Nervousness,  660 


Neuralgia,  intercostal  and  spinal  growths^ 

302 
Neurasthenia,  668 
Neurilemma,  62 
Neuritic  muscular  atrophy,  534,  593,  597 

(see  Peroneal  form). 
Neuritis,  82 

—  adventitial,  83 

—  after  acute  diseases,  147 

—  —  injuries,  80 

—  alcoholic,  150 

—  and  atrophic  paralysis,  426 

—  and  endarteritis,  152 

—  and  gout,  137,  147 

—  and  hysteria,  176 

—  and  inflammation,  4 

—  and  myelitis,  372 

—  and  pachymeningitis,  174 

—  and  poliomyelitis,  174 

—  and  tabes,  169,  481 

—  ataxic  form,  158 

—  atheromatous,  151 
brachial,  119 

—  cancerous,  82 

—  chronic,  85 

—  classification  of,  144 
^  crural,  133 

—  degenerative,  144 

—  diabetic,  144,  150 

—  diffuse,  84 

—  disseminated,  84 

—  endemic,  185 

—  face  in,  156 

—  focal  or  disseminated,  84 

—  gonorrbceal,  147 

—  herpetic,  196 

—  idiopatliic,  146,  151 

—  inco-ordination  in,  158 

—  in  infantile  paralysis,  400,  414 

—  in  leprosy,  192 

—  in  leucocythsemia,  85 

—  in  Morvan's  disease,  647 
•^  in  peroneal  atrophy,  595 

—  isolated,  in  acute  disease,  83 

—  lipomatous,  85 

—  malarial,  185 

—  memory  in,  152 
and   —  migrans,  67,  82,  85 

—  motor  form,  153 

—  multiple,  82,  138 

—  —  complications,  16S 

—  —  course,  161 


686 


liNDEX. 


Neuritis,  multiple,  diagnosis,  172 

—  —  pathological  anatomy,  164 

—  —  pathology,  169 

—  —  prognosis,  178 

—  —  symptoms,  152 

—  —  treatment,  181 

—  near  bedsores,  68, 83 

—  nystagmus  in,  157 

—  ocular  movements  in,  156 

—  optic,  in  ataxic  paraplegia,  606 

—  —  in  chlorosis,  341 

—  —  in  cord  disease,  269 

—  —  in  multiple  neuritis,  168 
■"-  —  in  myelitis,  362 

—  organismal,  146 

—  pain  in,  154 

—  parenchymatous,  82,  84 

—  peripheral,  138  (see  Multiple). 

—  proliferative,  85 

—  psychical  symptoms  in,  152 

—  radicular,  119,  303 

—  rheumatic,  82, 146,  149 

—  senile,  146 

—  sensory  form,  153 

—  septiesemic,  146, 148 

-—  spinal  cord  affection  in,  168, 176 

—  syphilitic,  85, 146 

—  tongue  in,  156 

—  toxsemic,  144 

—  toxic,  144 

—  treatment,  88 

—  tremor  in,  155 

—  trophic  changes  in,  160>  172 
^—  tubercular,  147 

—  varieties  of,  82,  144 
— -  vocal  cords  in,  156 
Neuro-fibroraa,  93 
Neuroglia,  development  of,  629 

—  structure  of,  57,  201 
Neurokeratin,  62,  202 
Neuroma,  90 

—  amputation,  93,  95 

—  amyelinic,  90 

—  false,  90 

—  multiple,  92,  609 

—  myelinic,  90 

»—  of  nerve-roots,  544 

—  plexiform,  91,  93,  94,  96 
Neuromatodes  elepbantiasii,  9S 
Neuromyositis,  134 

Neuron,  55 

—  cerebro-spinal,  218 


Neuron,  spino-muscular,  213 

Neuropathic  disposition  in  cord  disease,  277 

Neuropilema,  54 

Neurotabes,  175,  448  (see  Pseudo*tabeg) 

Nodes  of  Ranvier,  62 

Nuclei,  internodal,  63 

Nucleus  cuneatus,  219 

—  function  of,  59 

—  gracilis,  216,  219 

—  post-pyramidal,  219 
Nutrition,  changes  in,  127,  277 

—  influence  on,  of  cord,  244 
Nutritional  disease,  1 

of  cord,  2,  664,  668 

Nutritive  changes  in  muscles,  70 
in  skin,  28 

Nystagmus  in  ataxic  paraplegia,  506 

—  in  Friedreich's  disease,  521 

—  in  multiple  neuritis,  157 

—  in  progressive  muscular  atrophy,  542 

—  in  spastic  paraplegia,  495 

—  in  syringomyelia,  643 


Obstetrical  paralysis,  118 
Obturator  internus,  46 

—  nerve  paralysis,  116 
Ocular  symptoms  in  tabes,  456 
(Edema,  vaso-motor,  618 
Operation  for  tumour,  626 
Ophthalmoplegia,  457 
Opponens  pollicis,  44 

Optic  atrophy,  457 

—  —  in  ataxic  paraplegia,  506 

—  ^  in  family  spastic  paralysis,  508 
Optic  neuritis  in  chlorosis,  341 

—  —  in  multiple  neuritis,  163 

—  —  in  myelitis,  362 
Organic  disease  of  cord,  1 
Organismal  neuritis,  146 
Organisms  in  beri-beri,  186 

—  in  myelitis,  346, 347 
Oval  field  of  Flechsig,  218 
Overflow  incontinence,  246,  361 
Over*nse,  muscular  atrophy  from,  668 


Pachymeningitis,  174,  309,  666 

—  and  progressive  mnscolar  atrophy,  656 

—  cervical  hypertrophic,  326,  623 

—  cervical,  and  syringomyelia,  644 


INDEX. 


687 


Pachymeningitis,  chronic,  322 

—  external,  309 

—  internal  hremorrhagic,  309 
Pain,  conduction  of,  in  cord,  236 

—  in  cord  diseases,  260,  393 
tumours,  393,  615 

—  sensibility  to,  15,  236 

in  syringomyelia,  641 

Pains,  excentric  radiating,  260 
Palmar  fascia,  contraction  of,  41 
Palsy,  Erb's  type  of,  112,  565 

—  infantile,  396 

Paradoxical  muscular  contraction,  27 
Paraesthesia,  15 
Paralysis  and  paresis,  9 

—  acute  ascending,  371,  427 

—  agitans,  tremor  in,  12 

—  atrophic  spinal,  395,  424 

—  essential,  of  children,  396 

—  from  lessened  pressure,  453 

—  infantile,  396 

—  —  electricity  in,  421 

—  —  neuritis  in,  400,  414 

—  of  muscles,  33 

—  periodic,  598 

—  pseudo-hypertrophic,  564,  567 
Paramyotone,  ataxic,  606 

—  congenital,  605 
Paraplegia,  253 

—  ataxic,  503 

and  meningitis,  331 

—  chlorotic,  341 

—  congenital  spastic,  495 

—  dolorosa,  261,  301 

—  hereditary  spastic,  503 

—  primary  spastic,  489 

—  -^  —  and  ataxic,  499 

—  —  ^  family  form,  503 

—  —  —  infantile  form,  496 

—  simple  senile,  530 

Parasitic  tumours  of  cord,  608,  609 
Parenchymatous  inflammation,  4,  274 

—  myelitis,  366 

—  neuritis,  82,  84 
Paresis,  9 

Pathological  diagnosis  of  cord  disease,  274 

Pathology  and  etiology  of  cord  disease,  274 

Pectineus  muscle,  47 

Pectoralis  major,  37 

Pellagra,  511 

Pelvic  outflow,  248 

Perimeningitis,  309 


Perineuritis,  82,  83 
Perineurium,  63 
Periodic  paralysis,  598 
Peripachymeningitis,  309 
Peripheral  arteries  of  cord,  227 

—  nerves  in  tabes,  473,  480 

—  neuritis,  138  {see  Neuritis). 
Peroneal  nerve,  paralysis  of,  117 

—  type  of  atrophy,  534,  593 

—  —  combined  with  idiopathic,  597 
Peroneus  longus  muscle,  50,  232 

—  brevis  muscle,  52 

Phrenic  nerve,  diseases  of,  33,  96,  97 
Phthisis  and  peripheral  neuritis,  169 
Plantar  nerves,  diseases  of,  117 

reflex,  20 

Plexiform  neuroma,  91,  93,  94,  96 
Plexus,  lumbar,  114,  116 

—  sacral,  114,  116 
Polio-myelitis,  396 

—  chronic  and  subacute,  343,  386,  424 
PolysBsthesia,  14,  453 
Polymyositis,  acute,  185 
Polyneuritis,  82,  138,  173 

—  septicsemic,  146,  148 

—  tubercuhir,  147 

Popliteal  nerves,  diseases  of,  117 
Popliteus  muscle,  48 
Post-pyramidal  nucleus,  219 
Posterior  central  artery,  227 

—  column,  function  of,  215 

—  cornual  artery,  227 

—  ground  fibres,  207 

—  intermediate  septum,  200 

—  lateral  arteries,  227 

—  medial  artery,  227 

—  median  column,  200 

—  —  septum,  200 

—  nerve-roots  in  Tabes,  472 

—  pyramid  of  medulla,  219 

—  radicular  artery,  227 

—  root  fibres,  207,  225 
zone,  200 

—  spinal  vein,  227 

—  thoracic  nerve,  101 

—  vesicular  tract  in  tabes,  206,  471 
Postero-external  column,  200,  215,  219 
Postero-mediau  column,  200,  207,  215 
Posture,  adaptation  to,  241 

—  loss  of  sense  of,  18,  237,  259,  454 
Pott's  disease,  286 

Power,  measurement  of,  9 


688 


INDEX. 


Pressure  myelitis,  276, 853,  391 

■^  sense  of,  14 

Priapism,  263 

Primary  hsBmatomyelia,  437 

^  lateral  sclerosis,  489 

^  —  and  general  paralysis,  497 

—  —  and  hysteria,  501 

—  —  —  infantile,  495 

—  spastic  paraplegia,  489 
^  — -  —  family  form,  503 

—  —  —  infantile,  495 
Primitive  can-il  of  cord,  629 

—  fibrils,  55,  56,  62 

—  sheath,  62 

Progressive  muscular  atrophy,  531 

—  _  and  pachymeningitis,  556 

_  —  and  syringomyelia,  556 

—  .»  —  and  tabes,  466 
Pronators,  40 

—  nerve  centres  for,  232 
Protopathic  progressive  muscular  atrophy, 

632 
Protoplasmic  processes,  56 
Pseudo-hypertrophic  paralysis,  564,  667 
__  ^  mode  of  rising  in,  572 

—  —  varieties  of,  576 

—  tabes,  159,  173,  175,  372,  481 
Psoas  muscle,  46,  232 

Ptosis  in  tabes,  456 
Pulse  in  tabes,  462 
Pupil  in  cord  disease,  266 

—  in  myelitis,  362 

Pyramidal  fibres,  207,  209,  210,  255 
__  _  mode  of  ending  in  cord,  212 

—  tracts  in  progressive  muscular  atrophy, 

550 
Pyrexia  in  cord  disease,  263 
Pyriformis  muscle,  46 

Q 

Qnadrafcus  femoris  muscle,  46 
Quadriceps,  47 

—  centre  for,  232 

Qualitative  changes  in  irritability,  73 
Quantitative  changes  in  irritability,  73 


Eachialgia,  260 
Badiating  pains,  260 
Radicular  neuritis,  119,  303 
Railway  spine,  661 
Bamoa  y  Cajal,  53 


Ranvier,  nodes  of,  62 
Reaction,  normal,  73 

—  of  degeneration,  31,  32, 71 

—  —  in  infantile  palsy,  403 

—  —  mixed  form,  32,  393 
Rectal  crises,  462 
Rectum,  centre  lor,  245 
Rectus  femoris,  47 
Redecussation  in  cord,  211 
Red  softening  of  cord,  348 

Reflex  action,  19,  238,  240,  262,  263 

— <  —  cutaneous,  19 

^  —  in  nerve  injury,  79 

—  ^  muscular,  20 

—  —  two  kinds  of,  in  cord,  238 

—  arc,  19,  239 

—  centre,  19,  239 

—  paralysis,  665 

—  spasm,  264 
Reflexes,  deep,  20 

—  superficial,  19 

latent  period  of,  24 

Regeneration  of  fibres  in  cord,  358 

—  of  nerves,  69 

Regressive  period  in  infantile  palsy,  408 
Reinforcement,  21 

Representation  of  movements  in  cord,  230 
Resistance  in  nerve  phenomena,  54 
Resisting  power,  deficiency  in,  6 
Reticular  formation,  lateral,  202 

—  —  posterior,  203 
Rheumatic  neuritis,  82, 137, 145 
Rheumatism  of  dorsal  muscles,  320 
Rhomboids,  35 

Rigidity,  clasp-knife,  265,  493 

—  in  cord  diseases,  256 

—  in  progressive  muscular  atrophy,  641 
Rolando,  grey  substance  of,  206 
Romberg's  symptom,  449 

Root  pains,  260 

—  symptoms  in  compression,  393 

—  zone,  200 
Boot-fibres,  cause  of,  224 
Roots,  anterior,  224 

—  distribution  of,  225 

—  posterior,  225 

S 

Sacral  plexns,  diseases  of,  114, 116 
Sartorius  muscle,  232 
Scapular  muscles,  34,  231 


I>"DEX. 


689 


ScTimidt,  incision  of,  63 
Pcliultze,  Max,  observation  of,  55 
Schwann,  white  substance  of,  62 

—  sheath  of,  62 
Sciatica,  123 

Sciatic  nerve,  disease  of,  116 

Sclero<iactyla,  647 

Sclerosis,  amyotrophic  lateral,  531 

—  annular,  382 

—  combined  lateral  and  posterior,  503 

—  focal,  386 

—  from  anae'.rJa,  <fcc.,  513 

—  from  pe  laara,  511 

—  from  toxic  blocd-statea,  511 

—  in  chronic  mnscular  atrophy,  550 

—  insuLir,  4,  275 

—  lateral,  and  meuingitis,  323,  489 

—  secondary, 4 
Sclerotic  m\  elitis,  386 

Secondary  degeneration  of  cord,  208,  210, 

215 
ascending,  203,  215 

—  —  descending,  203, 210 

—  ^  of  nerres,  65 

—  tabes,  418 
SemimembranosTis,  47 
Semitendinosns,  47 
Sensation,  loca!isat"on  of,  13 

—  path  of,  233 

—  rhythmical  recurrence,  453 
Sensations,  spontaneous,  261 
Sense,  mnscular,  16 

—  of  pain,  15 

—  tactile,  13 

—  temperature,  15 
Sensibility,  modes  of  testing,  IS 

—  modidcatlons  of,  14 

—  muscular,  237 

—  recurrent,  73 

—  tactile,  13,  236,  238 

—  to  electricity,  15 

—  to  pain,  15,  236 

—  to  temperature,  15,  237 
S^-nsitiveness,  increased,  259 
Sensory  conduction  ia  cor.l,  233 

—  symptoms,  13,  78,  258 

—  —  in  nerve  lesions,  78 

in  tabes,  450 

Septicaemia  in  cord  disease*,  277 
Septicsemic  polyneuritis,  145 
Septomarginal  tract,  213,  220 
Septum,  posterior  intermediare,  200,  215 

VOL.   I. 


.^rptum.  posterior  median,  200 
Serratus  magnua,  35,  102,  231 

nerve  to,  101 

Sexual  functions,  centre  for,  248 

—  power  in  nutritional  diaeai.ae,  670 
in  tabes,  454 

in  tranmatie  lesioBs,  G63 

Sheath,  primitive,  62 

Shoulder  disloca  tion,  palsy  of  nerves  in,  1 11 

—  fracture.  111 

—  muscles  moving  the,  34 
Stin,  glossy,  28 

—  nutrition  of,  28,  245,  266 

—  representation  in  cord,  250,  251 
Sleep  palsies,  101 

Softening  of  cord,  348 

red.  348 

wL.te,  348 

yellow,  348 

Soleus  mnacle,  48 
Spasm,  11 

—  clonic,  11,  256 

—  in  cord  disease,  255 

—  in  meningitis,  317 

—  in  progressive  muscular  atrophy,  541 

—  reflex,  264 

—  tetanic,  13 

—  tonic,  11,  255 

Spastic  paralysis,  family  form,  503 

—  paraplegia,  eoiiirenital,  495 
in  caries,  291 

primary,  489 

Special  senses,  nature  of,  61 
Sphincter  ani,  centre  for,  245 
Sphincters  in  neuritis,  161 

—  in  taoes,  44S,  4oS 

—  paralysis  of,  245 — 247,  267 
Spider^lls,  201.  351 

Spina  bifida,  647 

arthropathy  in,  B52 

—  —  deformities  in,  '^52 

—  —  occulta,  r47 

—  —  trophic  changes  in,  6o2 
Spinal  column,  growths  in,  2v9 

—  card,  abscess  of,  377 

—  —  anemia  of,  339,  513 

—  —  anatomy  of,  200 

^  —  blood-supply  of,  226 

—  —  compression  of,  390 

degeneration  of,  442 

diseases  of,  199,  339 

—  —  embolism,  377 

44 


690 


INDEX. 


Spinal  cord,  functions  of,  228 
haemorrhage  into,  437 

—  —  hypersemia  of,  342 

inflammation  of,  342  (^see  Myelitis) 

softening  of,  275,  348 

structure  of,  200 

symptoms  of  disease  of,  253 

—  curvature  in  idiopathic  atrophy,  588 
in  infantile  palsy,  408 

—  —  in   progressive  muscular    atrophy, 

638 

—  —  in  pseudo-hypertrophy,  575 

—  epilepsj',  265 

—  hemiplegia,  272 

—  irritation,  295,  660 

—  membranes,  199 

—  meningeal  haemorrhage,  320 

—  meningitis,  309 

~-  muscular  atrophy,  531 

—  paralysis,  acute  atrophic,  396 

subacute,  chronic,  424 

Spindles,  muscle,  16,  671 
Spine,  caries  of,  286 

—  concussion  of,  652 

—  diseases  of,  281 
of  articulations  of,  307 

—  dislocation  of,  281 

—  fracture,  282 

—  gunshot  wounds  of,  652 

—  injuries  of,  281 

—  syphilitic  diseases  of,  305 

—  tumours  of,  299 
Spino-muscular  motor  segment,  213 
S|)ongioplasm,  56,  60 
Spongy  grey  substance,  203 

—  zone,  207 
Spontaneous  movements,  U 

—  —  in  tabes,  450 

—  sensations,  261 
Sterno-mastoid,  34 
Stretching  nerves  in  tahes,  489 
Structural  diseases,  2,  277 
Strychnia  in  muscular  atrophy,  557 
Subscapularis,  37 
Supinator  brevis,  40 

—  longus,  39 
Supinators,  231 

—  in  idiopathic  atrophy,  586 
Supra-scapular  nerve,  102 
Supra-spinatus,  37 
Suspension  in  tabes,  489 
Symptoms,  combination  of,  269 


Symptoms  in  cord  disease,  253 

—  mental,  8 

—  mode  of  investigation,  7 

—  motor,  8,  254 

—  sensory,  13,  78,  258 
Synergic  contraction,  10 

Syphilis  and  spinal  degenerations^  278, 
347,  380,  384 

—  and  injury  to  cord,  659 

—  and  tabes,  446 

—  in  Landry's  paralysis,  432 

—  in  progressive  muscular  atrophy,  534 
Syphilitic  caries,  305 

—  diseases  of  cord,  278 

—  meningitis,  330 

—  neuritis,  146 
Syphilomata  of  cord,  614 
Syringomyelia,  628 

—  and  cervical  pachymeningitis,  644 

—  and  haemorrhage,  644 

—  and  injuries,  639 

—  and  leprous  neuritis,  196,  647 

—  and  meningitis,  331 

—  and  myelitis,  384 

—  and  progressive  muscular  atrophy,  556, 

644 

—  and  tumours,  610,  613,  631,  644 

—  Charcot's  joint  in,  643 

—  motor  symptoms  in,  642 

—  muscular  atrophy  in,  642 

—  operation  in,  645 

—  pain  sense  in,  234,  260,  640 

—  sweating  in,  643 

—  temperature  sense  in,  234,  260,  640 
Syringo-m\  elocele,  650 

System  diseases,  253,  254,  274,  442 

T 

Tabes  dorsalis,  444 

and  alcoholism,  448 

and  ataxic  paraplegia,  466 

and  cerebellar  tumour,  483 

and  concussion,  447 

and  diphtheritic  paralysis,  483 

and  disease  of  posterior  roots,  478 

(foot-note) 

and  general  paralysis,  446 

and  myelitis,  448 

and  neuritis,  481 

and  soft  sores,  447 

and  syphilis,  466,  482 


INDEX. 


691 


Tabes  dorsalis  and  tumours  of  nerTe-roots, 

478 
'  Cliarcot's  joint  in,  459 

—  —  congenital,  651 
crises,  461, 462 

—  —  deafness  in,  457 
electricity  in,  488 

—  —  eye  symptoms  in,  445 
heart  disease  in,  466 

—  —  lightning  pains  in,  448 

Lissauer's  tract  in,  469 

motor  symptoms  in,  449 

—  —  ocular  symptoms  in,  445 

—  —  optic  atrophy  in,  457 
pathological  anatomy  of,  467 

—  —  pathology  of,  474 

—  —  reflex  action  in,  454 
— symptoms  in,  474 

—  —  secondary,  448 

—  —  sensory  symptoms  in,  450 

—  —  sexual  power  in,  454 

—  —  sphincters  in,  448,  458 
suspension  in,  489 

—  —  trophic  and  vaso»motor  symptoms 

in,  459 

visceral  symptoms  in,  461 

Tabetic  foot,  461 

—  multiple  neuritis,  448,  481 

—  neuralgia,  481 

—  ptosis,  456 

Tache  spinale,  318,  618 
Tactile  sensibility,  236 
Talipes  calcaneus,  50 

—  in  infantile  palsy,  406 

—  in  pseudo-hypertrophy,  574 

—  valfius,  51 

—  varus,  51 

Temperature  sense  in  syringomyelia,  234, 
260 

path  of,  15,  237,  640 

Tendon-reflex,  so-called,  19—21,  26 
Tensor  vaginse  femoris,  46 
Teres  major,  37 

—  minor,  37 

Tetanus  and  meningitis,  320 

—  neonatorum,  334 
Tetany,  nocturnal,  666 
Thenar  muscles,  44,  45 

centre  for,  232 

Thigh,  adductors  of,  47 

—  motor  points,  46,  49 

—  rotators,  46 


Thomsen's  disease,  601 

—  —  and  pseudo-hypertrophy,  576 
Thoracic  muscles,  33 

Thrilling  sensations,  15 
Thrombosis  in  cord  vessels,  276 
Thumb  muscles,  42 
Tibialis  anticus,  50,  232 

—  posticus,  52 
Tic  and  tabes,  451 
Tingling  sensations,  15,  81 
Toes,  muscles  moving,  52 
Tone,  muscular,  8,  12,  26 

Tonic  atrophy  of  muscles,  541,  553 

—  spasm,  11 

Torticollis,  muscles  affected  in,  34 

Touch,  sensibility  to,  13 

Toxic  influences  in  Landry's  paralysis,  432 

—  —  in  nervous  disease,  5,  277 

—  —  in  tabes,  5 

Tiact,  nntero-lateral  ascending,  208 
descending,  216 

—  comma-shaped,  216,  219 

—  cornu-commissural,  220 

—  direct  cerebellar,  207,  209,  220 

—  Gowers',  201,  208,  222,  470 

—  intermedio-lateral,  205 

—  Lissauer's,  221,  225,  226 

—  septo-uiarginal,  218,  220 
Tracts,  pyramidal,  207,  2U9,  210,  255 
Transverse  lesions  of  cord,  264,  269,  27 1 
kucf-jerk  in,  264 

—  myelitis,  365,  378 
Trapezius,  34 

Traumatic  lesions  of  cord,  652 

—  spinal  neuralgia,  660 
Tremor,  257 

—  contractions  in,  12 
Triceps,  37 

—  paralysis  of,  231 

Trophic  changes  in  myelitis.  362 
in  tabes,  459 

—  disorders,  28 

in  concussion,  655 

—  disturbance  in  cord  disease,  265 

—  nerves,  27,  28,  79 
Tubercula  dolorosa,  92,  95 
Tubercular  meningitis,  317 

—  polyneuritis,  147 

—  tumours  of  cord,  609,  619 
Tumours  of  cord,  291),  G07 
and  hysteria,  624 

and  membranes,  619,  622 


692 


INDEX. 


Tumours  of  cord,  surgical  treatment  of, 
626 

—  of  spine,  299 
—  and  caries,  303 

U 

Ulcer,  perforating,  459;  in  spina  bifula, 

652 
Ulnar  nerve,  disease  of,  108 
Ultimum  moriens,  536 
Unilateral  lesions  of  cor.l,  2^5,  272 

sensibility  in,  235,  272 

Unipolar  cell,  57 

Upper  arm  type  of  palsy,  407 

—  limb  muscles,  34;  nerves,  98 
Urethral  crises,  462 

Uterine  disease  and  cord  disease,  669 

—  functions,  centres  for,  248 


Vacuolation  of  cells,  351 
Vagus  in  peripheral  neuritis,  179 
Vascular  thickening  in  tabes,  471 
Vaso-niotor  centres,  248,  265,  266 

—  disorders,  79 

in  talies,  459 

Vasti  muscles,  47 
Veins  of  cord,  226 
Vertebras,  erosion  of,  305 
Vertebral  arthritis,  307 

—  column,  diseases  of,  281 

aneurism,  erosion  by,  305 

growths,  299 


Vertebral  column,  hydatids  of,  306 

syphilitic  disease  of,  305 

fracture  of,  282 

—  exostoses,  304 
Vesical  crises,  462 

—  control,  245 
Vessels  of  cord,  226 
Visceial  disturbance,  2 1 5,  267 

—  pains  in  tabes,  452,  4R1 

—  symptoms  in  tabes,  -IfJl 
Vitreous  degeneration,  70,  516 
Vocal  cords  in  tabes,  463 
Voltaism  in  diagnosis,  30 

W 

Waldeyer,  53 

Wallerian  degeneration,  65 

Wasting  palsy,  531 

Westpbal's  symptom,  407 

White  commissure  of  cord,  201,  223,  224 

—  softening,  348 

—  substance,  207 
Whitlows,  painless,  645 
Wrist  muscles,  40 

centre  for,  231,  238 


Yellow  softening,  348 

Z 

Zone,  spongy,  207 

Zygomatic  muscles  in  idiopathic  strophyt 
687 


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